Hereditary and sporadic conditions which are characterized by progressive nervous system dysfunction. These disorders are often associated with atrophy of the affected central or peripheral nervous system structures.
Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways.
A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)
The basic cellular units of nervous tissue. Each neuron consists of a body, an axon, and dendrites. Their purpose is to receive, conduct, and transmit impulses in the NERVOUS SYSTEM.
A degenerative disease of the BRAIN characterized by the insidious onset of DEMENTIA. Impairment of MEMORY, judgment, attention span, and problem solving skills are followed by severe APRAXIAS and a global loss of cognitive abilities. The condition primarily occurs after age 60, and is marked pathologically by severe cortical atrophy and the triad of SENILE PLAQUES; NEUROFIBRILLARY TANGLES; and NEUROPIL THREADS. (From Adams et al., Principles of Neurology, 6th ed, pp1049-57)
Microtubule-associated proteins that are mainly expressed in neurons. Tau proteins constitute several isoforms and play an important role in the assembly of tubulin monomers into microtubules and in maintaining the cytoskeleton and axonal transport. Aggregation of specific sets of tau proteins in filamentous inclusions is the common feature of intraneuronal and glial fibrillar lesions (NEUROFIBRILLARY TANGLES; NEUROPIL THREADS) in numerous neurodegenerative disorders (ALZHEIMER DISEASE; TAUOPATHIES).
A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)
The part of CENTRAL NERVOUS SYSTEM that is contained within the skull (CRANIUM). Arising from the NEURAL TUBE, the embryonic brain is comprised of three major parts including PROSENCEPHALON (the forebrain); MESENCEPHALON (the midbrain); and RHOMBENCEPHALON (the hindbrain). The developed brain consists of CEREBRUM; CEREBELLUM; and other structures in the BRAIN STEM.
A progressive, degenerative neurologic disease characterized by a TREMOR that is maximal at rest, retropulsion (i.e. a tendency to fall backwards), rigidity, stooped posture, slowness of voluntary movements, and a masklike facial expression. Pathologic features include loss of melanin containing neurons in the substantia nigra and other pigmented nuclei of the brainstem. LEWY BODIES are present in the substantia nigra and locus coeruleus but may also be found in a related condition (LEWY BODY DISEASE, DIFFUSE) characterized by dementia in combination with varying degrees of parkinsonism. (Adams et al., Principles of Neurology, 6th ed, p1059, pp1067-75)
A synuclein that is a major component of LEWY BODIES that plays a role in neurodegeneration and neuroprotection.
Neurodegenerative disorders involving deposition of abnormal tau protein isoforms (TAU PROTEINS) in neurons and glial cells in the brain. Pathological aggregations of tau proteins are associated with mutation of the tau gene on chromosome 17 in patients with ALZHEIMER DISEASE; DEMENTIA; PARKINSONIAN DISORDERS; progressive supranuclear palsy (SUPRANUCLEAR PALSY, PROGRESSIVE); and corticobasal degeneration.
Drugs intended to prevent damage to the brain or spinal cord from ischemia, stroke, convulsions, or trauma. Some must be administered before the event, but others may be effective for some time after. They act by a variety of mechanisms, but often directly or indirectly minimize the damage produced by endogenous excitatory amino acids.
Amino acid transporter systems capable of transporting acidic amino acids (AMINO ACIDS, ACIDIC).
A generic term for any circumscribed mass of foreign (e.g., lead or viruses) or metabolically inactive materials (e.g., ceroid or MALLORY BODIES), within the cytoplasm or nucleus of a cell. Inclusion bodies are in cells infected with certain filtrable viruses, observed especially in nerve, epithelial, or endothelial cells. (Stedman, 25th ed)
Inherited disorders characterized by progressive atrophy and dysfunction of anatomically or physiologically related neurologic systems.
Disorders caused by imbalances in the protein homeostasis network - synthesis, folding, and transport of proteins; post-translational modifications; and degradation or clearance of misfolded proteins.
The third type of glial cell, along with astrocytes and oligodendrocytes (which together form the macroglia). Microglia vary in appearance depending on developmental stage, functional state, and anatomical location; subtype terms include ramified, perivascular, ameboid, resting, and activated. Microglia clearly are capable of phagocytosis and play an important role in a wide spectrum of neuropathologies. They have also been suggested to act in several other roles including in secretion (e.g., of cytokines and neural growth factors), in immunological processing (e.g., antigen presentation), and in central nervous system development and remodeling.
Small proteinaceous infectious particles which resist inactivation by procedures that modify NUCLEIC ACIDS and contain an abnormal isoform of a cellular protein which is a major and necessary component. The abnormal (scrapie) isoform is PrPSc (PRPSC PROTEINS) and the cellular isoform PrPC (PRPC PROTEINS). The primary amino acid sequence of the two isoforms is identical. Human diseases caused by prions include CREUTZFELDT-JAKOB SYNDROME; GERSTMANN-STRAUSSLER SYNDROME; and INSOMNIA, FATAL FAMILIAL.
A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)
Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.
A family of homologous proteins of low MOLECULAR WEIGHT that are predominately expressed in the BRAIN and that have been implicated in a variety of human diseases. They were originally isolated from CHOLINERGIC FIBERS of TORPEDO.
Peptides generated from AMYLOID BETA-PEPTIDES PRECURSOR. An amyloid fibrillar form of these peptides is the major component of amyloid plaques found in individuals with Alzheimer's disease and in aged individuals with trisomy 21 (DOWN SYNDROME). The peptide is found predominantly in the nervous system, but there have been reports of its presence in non-neural tissue.
A group of dominantly inherited, predominately late-onset, cerebellar ataxias which have been divided into multiple subtypes based on clinical features and genetic mapping. Progressive ataxia is a central feature of these conditions, and in certain subtypes POLYNEUROPATHY; DYSARTHRIA; visual loss; and other disorders may develop. (From Joynt, Clinical Neurology, 1997, Ch65, pp 12-17; J Neuropathol Exp Neurol 1998 Jun;57(6):531-43)
Heterogeneous group of neurodegenerative disorders characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Multiple subtypes or forms are recognized based on presence or absence of TAU PROTEIN inclusions. FTLD includes three clinical syndromes: FRONTOTEMPORAL DEMENTIA, semantic dementia, and PRIMARY PROGRESSIVE NONFLUENT APHASIA.
The termination of the cell's ability to carry out vital functions such as metabolism, growth, reproduction, responsiveness, and adaptability.
A disturbance in the prooxidant-antioxidant balance in favor of the former, leading to potential damage. Indicators of oxidative stress include damaged DNA bases, protein oxidation products, and lipid peroxidation products (Sies, Oxidative Stress, 1991, pxv-xvi).
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
An increased number of contiguous trinucleotide repeats in the DNA sequence from one generation to the next. The presence of these regions is associated with diseases such as FRAGILE X SYNDROME and MYOTONIC DYSTROPHY. Some CHROMOSOME FRAGILE SITES are composed of sequences where trinucleotide repeat expansion occurs.
The most common clinical form of FRONTOTEMPORAL LOBAR DEGENERATION, this dementia presents with personality and behavioral changes often associated with disinhibition, apathy, and lack of insight.
An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75)
Semiautonomous, self-reproducing organelles that occur in the cytoplasm of all cells of most, but not all, eukaryotes. Each mitochondrion is surrounded by a double limiting membrane. The inner membrane is highly invaginated, and its projections are called cristae. Mitochondria are the sites of the reactions of oxidative phosphorylation, which result in the formation of ATP. They contain distinctive RIBOSOMES, transfer RNAs (RNA, TRANSFER); AMINO ACYL T RNA SYNTHETASES; and elongation and termination factors. Mitochondria depend upon genes within the nucleus of the cells in which they reside for many essential messenger RNAs (RNA, MESSENGER). Mitochondria are believed to have arisen from aerobic bacteria that established a symbiotic relationship with primitive protoeukaryotes. (King & Stansfield, A Dictionary of Genetics, 4th ed)
The segregation and degradation of damaged or unwanted cytoplasmic constituents by autophagic vacuoles (cytolysosomes) composed of LYSOSOMES containing cellular components in the process of digestion; it plays an important role in BIOLOGICAL METAMORPHOSIS of amphibians, in the removal of bone by osteoclasts, and in the degradation of normal cell components in nutritional deficiency states.
An acetamido cyclohexene that is a structural homolog of SIALIC ACID and inhibits NEURAMINIDASE.
An island in Micronesia, east of the Philippines, the largest and southernmost of the Marianas. Its capital is Agana. It was discovered by Magellan in 1521 and occupied by Spain in 1565. They ceded it to the United States in 1898. It is an unincorporated territory of the United States, administered by the Department of the Interior since 1950. The derivation of the name Guam is in dispute. (From Webster's New Geographical Dictionary, 1988, p471)
A fibrous protein complex that consists of proteins folded into a specific cross beta-pleated sheet structure. This fibrillar structure has been found as an alternative folding pattern for a variety of functional proteins. Deposits of amyloid in the form of AMYLOID PLAQUES are associated with a variety of degenerative diseases. The amyloid structure has also been found in a number of functional proteins that are unrelated to disease.
The gradual irreversible changes in structure and function of an organism that occur as a result of the passage of time.
An acquired organic mental disorder with loss of intellectual abilities of sufficient severity to interfere with social or occupational functioning. The dysfunction is multifaceted and involves memory, behavior, personality, judgment, attention, spatial relations, language, abstract thought, and other executive functions. The intellectual decline is usually progressive, and initially spares the level of consciousness.
Processes involved in the formation of TERTIARY PROTEIN STRUCTURE.
Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.
Abnormal structures located in various parts of the brain and composed of dense arrays of paired helical filaments (neurofilaments and microtubules). These double helical stacks of transverse subunits are twisted into left-handed ribbon-like filaments that likely incorporate the following proteins: (1) the intermediate filaments: medium- and high-molecular-weight neurofilaments; (2) the microtubule-associated proteins map-2 and tau; (3) actin; and (4) UBIQUITINS. As one of the hallmarks of ALZHEIMER DISEASE, the neurofibrillary tangles eventually occupy the whole of the cytoplasm in certain classes of cell in the neocortex, hippocampus, brain stem, and diencephalon. The number of these tangles, as seen in post mortem histology, correlates with the degree of dementia during life. Some studies suggest that tangle antigens leak into the systemic circulation both in the course of normal aging and in cases of Alzheimer disease.
A neurodegenerative disease characterized by dementia, mild parkinsonism, and fluctuations in attention and alertness. The neuropsychiatric manifestations tend to precede the onset of bradykinesia, MUSCLE RIGIDITY, and other extrapyramidal signs. DELUSIONS and visual HALLUCINATIONS are relatively frequent in this condition. Histologic examination reveals LEWY BODIES in the CEREBRAL CORTEX and BRAIN STEM. SENILE PLAQUES and other pathologic features characteristic of ALZHEIMER DISEASE may also be present. (From Neurology 1997;48:376-380; Neurology 1996;47:1113-1124)
A class of large neuroglial (macroglial) cells in the central nervous system - the largest and most numerous neuroglial cells in the brain and spinal cord. Astrocytes (from "star" cells) are irregularly shaped with many long processes, including those with "end feet" which form the glial (limiting) membrane and directly and indirectly contribute to the BLOOD-BRAIN BARRIER. They regulate the extracellular ionic and chemical environment, and "reactive astrocytes" (along with MICROGLIA) respond to injury.
Members of the class of compounds composed of AMINO ACIDS joined together by peptide bonds between adjacent amino acids into linear, branched or cyclical structures. OLIGOPEPTIDES are composed of approximately 2-12 amino acids. Polypeptides are composed of approximately 13 or more amino acids. PROTEINS are linear polypeptides that are normally synthesized on RIBOSOMES.
Toxic substances from microorganisms, plants or animals that interfere with the functions of the nervous system. Most venoms contain neurotoxic substances. Myotoxins are included in this concept.
A group of disorders which feature impaired motor control characterized by bradykinesia, MUSCLE RIGIDITY; TREMOR; and postural instability. Parkinsonian diseases are generally divided into primary parkinsonism (see PARKINSON DISEASE), secondary parkinsonism (see PARKINSON DISEASE, SECONDARY) and inherited forms. These conditions are associated with dysfunction of dopaminergic or closely related motor integration neuronal pathways in the BASAL GANGLIA.
An oxidoreductase that catalyzes the reaction between superoxide anions and hydrogen to yield molecular oxygen and hydrogen peroxide. The enzyme protects the cell against dangerous levels of superoxide. EC 1.15.1.1.
Theoretical representations that simulate the behavior or activity of biological processes or diseases. For disease models in living animals, DISEASE MODELS, ANIMAL is available. Biological models include the use of mathematical equations, computers, and other electronic equipment.
Intracytoplasmic, eosinophilic, round to elongated inclusions found in vacuoles of injured or fragmented neurons. The presence of Lewy bodies is the histological marker of the degenerative changes in LEWY BODY DISEASE and PARKINSON DISEASE but they may be seen in other neurological conditions. They are typically found in the substantia nigra and locus coeruleus but they are also seen in the basal forebrain, hypothalamic nuclei, and neocortex.
The directed transport of ORGANELLES and molecules along nerve cell AXONS. Transport can be anterograde (from the cell body) or retrograde (toward the cell body). (Alberts et al., Molecular Biology of the Cell, 3d ed, pG3)
The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges.
Microsatellite repeats consisting of three nucleotides dispersed in the euchromatic arms of chromosomes.
Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)
A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7)
Diseases caused by abnormal function of the MITOCHONDRIA. They may be caused by mutations, acquired or inherited, in mitochondrial DNA or in nuclear genes that code for mitochondrial components. They may also be the result of acquired mitochondria dysfunction due to adverse effects of drugs, infections, or other environmental causes.
Neurons which activate MUSCLE CELLS.
A highly conserved 76-amino acid peptide universally found in eukaryotic cells that functions as a marker for intracellular PROTEIN TRANSPORT and degradation. Ubiquitin becomes activated through a series of complicated steps and forms an isopeptide bond to lysine residues of specific proteins within the cell. These "ubiquitinated" proteins can be recognized and degraded by proteosomes or be transported to specific compartments within the cell.
Diseases of any component of the brain (including the cerebral hemispheres, diencephalon, brain stem, and cerebellum) or the spinal cord.
A curved elevation of GRAY MATTER extending the entire length of the floor of the TEMPORAL HORN of the LATERAL VENTRICLE (see also TEMPORAL LOBE). The hippocampus proper, subiculum, and DENTATE GYRUS constitute the hippocampal formation. Sometimes authors include the ENTORHINAL CORTEX in the hippocampal formation.
A plant genus of the family Cycadaceae, order Cycadales, class Cycadopsida, division CYCADOPHYTA of palm-like trees. It is a source of CYCASIN, the beta-D-glucoside of methylazoxymethanol.
Proteins that specifically bind to IRON.
A large multisubunit complex that plays an important role in the degradation of most of the cytosolic and nuclear proteins in eukaryotic cells. It contains a 700-kDa catalytic sub-complex and two 700-kDa regulatory sub-complexes. The complex digests ubiquitinated proteins and protein activated via ornithine decarboxylase antizyme.
A group of disorders marked by progressive degeneration of motor neurons in the spinal cord resulting in weakness and muscular atrophy, usually without evidence of injury to the corticospinal tracts. Diseases in this category include Werdnig-Hoffmann disease and later onset SPINAL MUSCULAR ATROPHIES OF CHILDHOOD, most of which are hereditary. (Adams et al., Principles of Neurology, 6th ed, p1089)
The thin layer of GRAY MATTER on the surface of the CEREBRAL HEMISPHERES that develops from the TELENCEPHALON and folds into gyri and sulchi. It reaches its highest development in humans and is responsible for intellectual faculties and higher mental functions.
A rare form of DEMENTIA that is sometimes familial. Clinical features include APHASIA; APRAXIA; CONFUSION; ANOMIA; memory loss; and personality deterioration. This pattern is consistent with the pathologic findings of circumscribed atrophy of the poles of the FRONTAL LOBE and TEMPORAL LOBE. Neuronal loss is maximal in the HIPPOCAMPUS, entorhinal cortex, and AMYGDALA. Some ballooned cortical neurons contain argentophylic (Pick) bodies. (From Brain Pathol 1998 Apr;8(2):339-54; Adams et al., Principles of Neurology, 6th ed, pp1057-9)
The intracellular transfer of information (biological activation/inhibition) through a signal pathway. In each signal transduction system, an activation/inhibition signal from a biologically active molecule (hormone, neurotransmitter) is mediated via the coupling of a receptor/enzyme to a second messenger system or to an ion channel. Signal transduction plays an important role in activating cellular functions, cell differentiation, and cell proliferation. Examples of signal transduction systems are the GAMMA-AMINOBUTYRIC ACID-postsynaptic receptor-calcium ion channel system, the receptor-mediated T-cell activation pathway, and the receptor-mediated activation of phospholipases. Those coupled to membrane depolarization or intracellular release of calcium include the receptor-mediated activation of cytotoxic functions in granulocytes and the synaptic potentiation of protein kinase activation. Some signal transduction pathways may be part of larger signal transduction pathways; for example, protein kinase activation is part of the platelet activation signal pathway.
The span of viability of a cell characterized by the capacity to perform certain functions such as metabolism, growth, reproduction, some form of responsiveness, and adaptability.
A multifunctional heterogeneous-nuclear ribonucleoprotein that may play a role in homologous DNA pairing and recombination. The N-terminal portion of protein is a potent transcriptional activator, while the C terminus is required for RNA binding. The name FUS refers to the fact that genetic recombination events result in fusion oncogene proteins (ONCOGENE PROTEINS, FUSION) that contain the N-terminal region of this protein. These fusion proteins have been found in myxoid liposarcoma (LIPOSARCOMA, MYXOID) and acute myeloid leukemia.
The non-neuronal cells of the nervous system. They not only provide physical support, but also respond to injury, regulate the ionic and chemical composition of the extracellular milieu, participate in the BLOOD-BRAIN BARRIER and BLOOD-RETINAL BARRIER, form the myelin insulation of nervous pathways, guide neuronal migration during development, and exchange metabolites with neurons. Neuroglia have high-affinity transmitter uptake systems, voltage-dependent and transmitter-gated ion channels, and can release transmitters, but their role in signaling (as in many other functions) is unclear.
The production of a dense fibrous network of neuroglia; includes astrocytosis, which is a proliferation of astrocytes in the area of a degenerative lesion.
One of the mechanisms by which CELL DEATH occurs (compare with NECROSIS and AUTOPHAGOCYTOSIS). Apoptosis is the mechanism responsible for the physiological deletion of cells and appears to be intrinsically programmed. It is characterized by distinctive morphologic changes in the nucleus and cytoplasm, chromatin cleavage at regularly spaced sites, and the endonucleolytic cleavage of genomic DNA; (DNA FRAGMENTATION); at internucleosomal sites. This mode of cell death serves as a balance to mitosis in regulating the size of animal tissues and in mediating pathologic processes associated with tumor growth.
Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control (induction or repression) of gene action at the level of transcription or translation.
Diseases of the central and peripheral nervous system. This includes disorders of the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscle.
Accumulations of extracellularly deposited AMYLOID FIBRILS within tissues.
Molecules or ions formed by the incomplete one-electron reduction of oxygen. These reactive oxygen intermediates include SINGLET OXYGEN; SUPEROXIDES; PEROXIDES; HYDROXYL RADICAL; and HYPOCHLOROUS ACID. They contribute to the microbicidal activity of PHAGOCYTES, regulation of signal transduction and gene expression, and the oxidative damage to NUCLEIC ACIDS; PROTEINS; and LIPIDS.
A syndrome complex composed of three conditions which represent clinical variants of the same disease process: STRIATONIGRAL DEGENERATION; SHY-DRAGER SYNDROME; and the sporadic form of OLIVOPONTOCEREBELLAR ATROPHIES. Clinical features include autonomic, cerebellar, and basal ganglia dysfunction. Pathologic examination reveals atrophy of the basal ganglia, cerebellum, pons, and medulla, with prominent loss of autonomic neurons in the brain stem and spinal cord. (From Adams et al., Principles of Neurology, 6th ed, p1076; Baillieres Clin Neurol 1997 Apr;6(1):187-204; Med Clin North Am 1999 Mar;83(2):381-92)
Proteins found in the nucleus of a cell. Do not confuse with NUCLEOPROTEINS which are proteins conjugated with nucleic acids, that are not necessarily present in the nucleus.
ANIMALS whose GENOME has been altered by GENETIC ENGINEERING, or their offspring.
The black substance in the ventral midbrain or the nucleus of cells containing the black substance. These cells produce DOPAMINE, an important neurotransmitter in regulation of the sensorimotor system and mood. The dark colored MELANIN is a by-product of dopamine synthesis.
A family of cellular proteins that mediate the correct assembly or disassembly of polypeptides and their associated ligands. Although they take part in the assembly process, molecular chaperones are not components of the final structures.
Abnormal isoform of prion proteins (PRIONS) resulting from a posttranslational modification of the cellular prion protein (PRPC PROTEINS). PrPSc are disease-specific proteins seen in certain human and animal neurodegenerative diseases (PRION DISEASES).
Histochemical localization of immunoreactive substances using labeled antibodies as reagents.
A synuclein that is closely related to ALPHA-SYNUCLEIN. It may play a neuroprotective role against some of the toxic effects of aggregated ALPHA-SYNUCLEIN.
A performance test based on forced MOTOR ACTIVITY on a rotating rod, usually by a rodent. Parameters include the riding time (seconds) or endurance. Test is used to evaluate balance and coordination of the subjects, particular in experimental animal models for neurological disorders and drug effects.
Conditions which feature clinical manifestations resembling primary Parkinson disease that are caused by a known or suspected condition. Examples include parkinsonism caused by vascular injury, drugs, trauma, toxin exposure, neoplasms, infections and degenerative or hereditary conditions. Clinical features may include bradykinesia, rigidity, parkinsonian gait, and masked facies. In general, tremor is less prominent in secondary parkinsonism than in the primary form. (From Joynt, Clinical Neurology, 1998, Ch38, pp39-42)
Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.
Circumscribed masses of foreign or metabolically inactive materials, within the CELL NUCLEUS. Some are VIRAL INCLUSION BODIES.
An increase number of repeats of a genomic, tandemly repeated DNA sequence from one generation to the next.
A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.
Neurons whose primary neurotransmitter is DOPAMINE.
Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes.
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
Striped GRAY MATTER and WHITE MATTER consisting of the NEOSTRIATUM and paleostriatum (GLOBUS PALLIDUS). It is located in front of and lateral to the THALAMUS in each cerebral hemisphere. The gray substance is made up of the CAUDATE NUCLEUS and the lentiform nucleus (the latter consisting of the GLOBUS PALLIDUS and PUTAMEN). The WHITE MATTER is the INTERNAL CAPSULE.
Diseases characterized by the presence of abnormally phosphorylated, ubiquitinated, and cleaved DNA-binding protein TDP-43 in affected brain and spinal cord. Inclusions of the pathologic protein in neurons and glia, without the presence of AMYLOID, is the major feature of these conditions, thus making these proteinopathies distinct from most other neurogenerative disorders in which protein misfolding leads to brain amyloidosis. Both frontotemporal lobar degeneration and AMYOTROPHIC LATERAL SCLEROSIS exhibit this common method of pathogenesis and thus they may represent two extremes of a continuous clinicopathological spectrum of one disease.
Naturally occurring or synthetic substances that inhibit or retard the oxidation of a substance to which it is added. They counteract the harmful and damaging effects of oxidation in animal tissues.
A serine-threonine kinase that plays important roles in CELL DIFFERENTIATION; CELL MIGRATION; and CELL DEATH of NERVE CELLS. It is closely related to other CYCLIN-DEPENDENT KINASES but does not seem to participate in CELL CYCLE regulation.
The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.
Type III intermediate filament proteins that assemble into neurofilaments, the major cytoskeletal element in nerve axons and dendrites. They consist of three distinct polypeptides, the neurofilament triplet. Types I, II, and IV intermediate filament proteins form other cytoskeletal elements such as keratins and lamins. It appears that the metabolism of neurofilaments is disturbed in Alzheimer's disease, as indicated by the presence of neurofilament epitopes in the neurofibrillary tangles, as well as by the severe reduction of the expression of the gene for the light neurofilament subunit of the neurofilament triplet in brains of Alzheimer's patients. (Can J Neurol Sci 1990 Aug;17(3):302)
A single-pass type I membrane protein. It is cleaved by AMYLOID PRECURSOR PROTEIN SECRETASES to produce peptides of varying amino acid lengths. A 39-42 amino acid peptide, AMYLOID BETA-PEPTIDES is a principal component of the extracellular amyloid in SENILE PLAQUES.
A nonspecific term referring both to the pathologic finding of swelling of distal portions of axons in the brain and to disorders which feature this finding. Neuroaxonal dystrophy is seen in various genetic diseases, vitamin deficiencies, and aging. Infantile neuroaxonal dystrophy is an autosomal recessive disease characterized by arrested psychomotor development at 6 months to 2 years of age, ataxia, brain stem dysfunction, and quadriparesis. Juvenile and adult forms also occur. Pathologic findings include brain atrophy and widespread accumulation of axonal spheroids throughout the neuroaxis, peripheral nerves, and dental pulp. (From Davis & Robertson, Textbook of Neuropathology, 2nd ed, p927)
Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or PERIPHERAL NERVE DISEASES. Motor ataxia may be associated with CEREBELLAR DISEASES; CEREBRAL CORTEX diseases; THALAMIC DISEASES; BASAL GANGLIA DISEASES; injury to the RED NUCLEUS; and other conditions.
Established cell cultures that have the potential to propagate indefinitely.
Inflammation of the BRAIN due to infection, autoimmune processes, toxins, and other conditions. Viral infections (see ENCEPHALITIS, VIRAL) are a relatively frequent cause of this condition.
Normal cellular isoform of prion proteins (PRIONS) encoded by a chromosomal gene and found in normal and scrapie-infected brain tissue, and other normal tissue. PrPC are protease-sensitive proteins whose function is unknown. Posttranslational modification of PrPC into PrPSC leads to infectivity.
Traumatic injuries to the brain, cranial nerves, spinal cord, autonomic nervous system, or neuromuscular system, including iatrogenic injuries induced by surgical procedures.
A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS. (From N Engl J Med, 1998 Dec 31;339(27))
The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills.
Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
An intermediate filament protein found only in glial cells or cells of glial origin. MW 51,000.
A dominantly-inherited ATAXIA first described in people of Azorean and Portuguese descent, and subsequently identified in Brazil, Japan, China, and Australia. This disorder is classified as one of the SPINOCEREBELLAR ATAXIAS (Type 3) and has been associated with a mutation of the MJD1 gene on chromosome 14. Clinical features include progressive ataxia, DYSARTHRIA, postural instability, nystagmus, eyelid retraction, and facial FASCICULATIONS. DYSTONIA is prominent in younger patients (referred to as Type I Machado-Joseph Disease). Type II features ataxia and ocular signs; Type III features MUSCULAR ATROPHY and a sensorimotor neuropathy; and Type IV features extrapyramidal signs combined with a sensorimotor neuropathy. (From Clin Neurosci 1995;3(1):17-22; Ann Neurol 1998 Mar;43(3):288-96)
A class of morphologically heterogeneous cytoplasmic particles in animal and plant tissues characterized by their content of hydrolytic enzymes and the structure-linked latency of these enzymes. The intracellular functions of lysosomes depend on their lytic potential. The single unit membrane of the lysosome acts as a barrier between the enzymes enclosed in the lysosome and the external substrate. The activity of the enzymes contained in lysosomes is limited or nil unless the vesicle in which they are enclosed is ruptured. Such rupture is supposed to be under metabolic (hormonal) control. (From Rieger et al., Glossary of Genetics: Classical and Molecular, 5th ed)
Compounds having the nitro group, -NO2, attached to carbon. When attached to nitrogen they are nitramines and attached to oxygen they are NITRATES.
Proteins that form the core of amyloid fibrils. For example, the core of amyloid A is formed from amyloid A protein, also known as serum amyloid A protein or SAA protein.
One of the catecholamine NEUROTRANSMITTERS in the brain. It is derived from TYROSINE and is the precursor to NOREPINEPHRINE and EPINEPHRINE. Dopamine is a major transmitter in the extrapyramidal system of the brain, and important in regulating movement. A family of receptors (RECEPTORS, DOPAMINE) mediate its action.
An active neurotoxic metabolite of 1-METHYL-4-PHENYL-1,2,3,6-TETRAHYDROPYRIDINE. The compound reduces dopamine levels, inhibits the biosynthesis of catecholamines, depletes cardiac norepinephrine and inactivates tyrosine hydroxylase. These and other toxic effects lead to cessation of oxidative phosphorylation, ATP depletion, and cell death. The compound, which is related to PARAQUAT, has also been used as an herbicide.
The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.
Formation of NEURONS which involves the differentiation and division of STEM CELLS in which one or both of the daughter cells become neurons.
Disturbances in mental processes related to learning, thinking, reasoning, and judgment.
Biphasic dose responses of cells or organisms (including microorganisms) to an exogenous or intrinsic factor, in which the factor induces stimulatory or beneficial effects at low doses and inhibitory or adverse effects at high doses.
Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM.
A heterogenous group of degenerative syndromes marked by progressive cerebellar dysfunction either in isolation or combined with other neurologic manifestations. Sporadic and inherited subtypes occur. Inheritance patterns include autosomal dominant, autosomal recessive, and X-linked.
Neurologic disorders caused by exposure to toxic substances through ingestion, injection, cutaneous application, or other method. This includes conditions caused by biologic, chemical, and pharmaceutical agents.
Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.
A fatal disease of the nervous system in sheep and goats, characterized by pruritus, debility, and locomotor incoordination. It is caused by proteinaceous infectious particles called PRIONS.
The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.
Degeneration of distal aspects of a nerve axon following injury to the cell body or proximal portion of the axon. The process is characterized by fragmentation of the axon and its MYELIN SHEATH.
A homolog of ALPHA-SYNUCLEIN that plays a role in neurofilament network integrity. It is overexpressed in a variety of human NEOPLASMS and may be involved in modulating AXON architecture during EMBRYONIC DEVELOPMENT and in the adult. Gamma-Synuclein may also activate SIGNAL TRANSDUCTION PATHWAYS associated with ETS-DOMAIN PROTEIN ELK-1.
A dopaminergic neurotoxic compound which produces irreversible clinical, chemical, and pathological alterations that mimic those found in Parkinson disease.
Changes in the amounts of various chemicals (neurotransmitters, receptors, enzymes, and other metabolites) specific to the area of the central nervous system contained within the head. These are monitored over time, during sensory stimulation, or under different disease states.
A CELL LINE derived from a PHEOCHROMOCYTOMA of the rat ADRENAL MEDULLA. PC12 cells stop dividing and undergo terminal differentiation when treated with NERVE GROWTH FACTOR, making the line a useful model system for NERVE CELL differentiation.
Proteins produced from GENES that have acquired MUTATIONS.
High molecular weight proteins found in the MICROTUBULES of the cytoskeletal system. Under certain conditions they are required for TUBULIN assembly into the microtubules and stabilize the assembled microtubules.
Proteins encoded by the mitochondrial genome or proteins encoded by the nuclear genome that are imported to and resident in the MITOCHONDRIA.
The process of moving proteins from one cellular compartment (including extracellular) to another by various sorting and transport mechanisms such as gated transport, protein translocation, and vesicular transport.
A neurotransmitter analogue that depletes noradrenergic stores in nerve endings and induces a reduction of dopamine levels in the brain. Its mechanism of action is related to the production of cytolytic free-radicals.
A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.
An autosomal recessive lipid storage disorder that is characterized by accumulation of CHOLESTEROL and SPHINGOMYELINS in cells of the VISCERA and the CENTRAL NERVOUS SYSTEM. Type C (or C1) and type D are allelic disorders caused by mutation of gene (NPC1) encoding a protein that mediate intracellular cholesterol transport from lysosomes. Clinical signs include hepatosplenomegaly and chronic neurological symptoms. Type D is a variant in people with a Nova Scotia ancestry.
Protein analogs and derivatives of the Aequorea victoria green fluorescent protein that emit light (FLUORESCENCE) when excited with ULTRAVIOLET RAYS. They are used in REPORTER GENES in doing GENETIC TECHNIQUES. Numerous mutants have been made to emit other colors or be sensitive to pH.
A strain of albino rat used widely for experimental purposes because of its calmness and ease of handling. It was developed by the Sprague-Dawley Animal Company.
Peptide hydrolases that contain at the active site a SERINE residue involved in catalysis.
A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.
An autosomal dominant disorder characterized by degeneration of the THALAMUS and progressive insomnia. It is caused by a mutation in the prion protein (PRIONS).
A genus of small, two-winged flies containing approximately 900 described species. These organisms are the most extensively studied of all genera from the standpoint of genetics and cytology.
A member of the nerve growth factor family of trophic factors. In the brain BDNF has a trophic action on retinal, cholinergic, and dopaminergic neurons, and in the peripheral nervous system it acts on both motor and sensory neurons. (From Kendrew, The Encyclopedia of Molecular Biology, 1994)
Specialized non-fenestrated tightly-joined ENDOTHELIAL CELLS with TIGHT JUNCTIONS that form a transport barrier for certain substances between the cerebral capillaries and the BRAIN tissue.
Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.
The introduction of a phosphoryl group into a compound through the formation of an ester bond between the compound and a phosphorus moiety.
The continuous remodeling of MITOCHONDRIA shape by fission and fusion in response to physiological conditions.
The use of silver, usually silver nitrate, as a reagent for producing contrast or coloration in tissue specimens.
A botanical insecticide that is an inhibitor of mitochondrial electron transport.
A statistical technique that isolates and assesses the contributions of categorical independent variables to variation in the mean of a continuous dependent variable.
The observable response an animal makes to any situation.
Elements of limited time intervals, contributing to particular results or situations.
A group of inherited diseases that share similar phenotypes but are genetically diverse. Different genetic loci for autosomal recessive, autosomal dominant, and x-linked forms of hereditary spastic paraplegia have been identified. Clinically, patients present with slowly progressive distal limb weakness and lower extremity spasticity. Peripheral sensory neurons may be affected in the later stages of the disease. (J Neurol Neurosurg Psychiatry 1998 Jan;64(1):61-6; Curr Opin Neurol 1997 Aug;10(4):313-8)
The process by which chemical compounds provide protection to cells against harmful agents.
The assembly of the QUATERNARY PROTEIN STRUCTURE of multimeric proteins (MULTIPROTEIN COMPLEXES) from their composite PROTEIN SUBUNITS.
RNA sequences that serve as templates for protein synthesis. Bacterial mRNAs are generally primary transcripts in that they do not require post-transcriptional processing. Eukaryotic mRNA is synthesized in the nucleus and must be exported to the cytoplasm for translation. Most eukaryotic mRNAs have a sequence of polyadenylic acid at the 3' end, referred to as the poly(A) tail. The function of this tail is not known for certain, but it may play a role in the export of mature mRNA from the nucleus as well as in helping stabilize some mRNA molecules by retarding their degradation in the cytoplasm.
Self-renewing cells that generate the main phenotypes of the nervous system in both the embryo and adult. Neural stem cells are precursors to both NEURONS and NEUROGLIA.
Cleavage of proteins into smaller peptides or amino acids either by PROTEASES or non-enzymatically (e.g., Hydrolysis). It does not include Protein Processing, Post-Translational.
Electron microscopy in which the ELECTRONS or their reaction products that pass down through the specimen are imaged below the plane of the specimen.
The physical activity of a human or an animal as a behavioral phenomenon.
The output neurons of the cerebellar cortex.
A condition caused by the neurotoxin MPTP which causes selective destruction of nigrostriatal dopaminergic neurons. Clinical features include irreversible parkinsonian signs including rigidity and bradykinesia (PARKINSON DISEASE, SECONDARY). MPTP toxicity is also used as an animal model for the study of PARKINSON DISEASE. (Adams et al., Principles of Neurology, 6th ed, p1072; Neurology 1986 Feb;36(2):250-8)
The worsening of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.
A state in which attention is largely directed inward upon one's self.
A mutation in which a codon is mutated to one directing the incorporation of a different amino acid. This substitution may result in an inactive or unstable product. (From A Dictionary of Genetics, King & Stansfield, 5th ed)
A subclass of histone deacetylases that are NAD-dependent. Several members of the SIRTUINS family are included in this subclass.
Specialized junctions at which a neuron communicates with a target cell. At classical synapses, a neuron's presynaptic terminal releases a chemical transmitter stored in synaptic vesicles which diffuses across a narrow synaptic cleft and activates receptors on the postsynaptic membrane of the target cell. The target may be a dendrite, cell body, or axon of another neuron, or a specialized region of a muscle or secretory cell. Neurons may also communicate via direct electrical coupling with ELECTRICAL SYNAPSES. Several other non-synaptic chemical or electric signal transmitting processes occur via extracellular mediated interactions.
The characteristic 3-dimensional shape and arrangement of multimeric proteins (aggregates of more than one polypeptide chain).
Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.
A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).
Proteins which bind to DNA. The family includes proteins which bind to both double- and single-stranded DNA and also includes specific DNA binding proteins in serum which can be used as markers for malignant diseases.
Linear POLYPEPTIDES that are synthesized on RIBOSOMES and may be further modified, crosslinked, cleaved, or assembled into complex proteins with several subunits. The specific sequence of AMINO ACIDS determines the shape the polypeptide will take, during PROTEIN FOLDING, and the function of the protein.
A species of fruit fly much used in genetics because of the large size of its chromosomes.
A cell line generated from human embryonic kidney cells that were transformed with human adenovirus type 5.
A diverse class of enzymes that interact with UBIQUITIN-CONJUGATING ENZYMES and ubiquitination-specific protein substrates. Each member of this enzyme group has its own distinct specificity for a substrate and ubiquitin-conjugating enzyme. Ubiquitin-protein ligases exist as both monomeric proteins multiprotein complexes.
The middle of the three primitive cerebral vesicles of the embryonic brain. Without further subdivision, midbrain develops into a short, constricted portion connecting the PONS and the DIENCEPHALON. Midbrain contains two major parts, the dorsal TECTUM MESENCEPHALI and the ventral TEGMENTUM MESENCEPHALI, housing components of auditory, visual, and other sensorimoter systems.
In tissue culture, hairlike projections of neurons stimulated by growth factors and other molecules. These projections may go on to form a branched tree of dendrites or a single axon or they may be reabsorbed at a later stage of development. "Neurite" may refer to any filamentous or pointed outgrowth of an embryonal or tissue-culture neural cell.
Tests designed to assess neurological function associated with certain behaviors. They are used in diagnosing brain dysfunction or damage and central nervous system disorders or injury.
A metabolite of tryptophan with a possible role in neurodegenerative disorders. Elevated CSF levels of quinolinic acid are correlated with the severity of neuropsychological deficits in patients who have AIDS.
A division of GYMNOSPERMS which look like palm trees (ARECACEAE) but are more closely related to PINUS. They have large cones and large pinnate leaves and are sometimes called cycads, a term which may also refer more narrowly to cycadales or CYCAS.
A species of nematode that is widely used in biological, biochemical, and genetic studies.
The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.
The transfer of STEM CELLS from one individual to another within the same species (TRANSPLANTATION, HOMOLOGOUS) or between species (XENOTRANSPLANTATION), or transfer within the same individual (TRANSPLANTATION, AUTOLOGOUS). The source and location of the stem cells determines their potency or pluripotency to differentiate into various cell types.
The phenotypic manifestation of a gene or genes by the processes of GENETIC TRANSCRIPTION and GENETIC TRANSLATION.
Proteins that originate from insect species belonging to the genus DROSOPHILA. The proteins from the most intensely studied species of Drosophila, DROSOPHILA MELANOGASTER, are the subject of much interest in the area of MORPHOGENESIS and development.
An enzyme that catalyzes the conversion of L-tyrosine, tetrahydrobiopterin, and oxygen to 3,4-dihydroxy-L-phenylalanine, dihydrobiopterin, and water. EC 1.14.16.2.
A class of large neuroglial (macroglial) cells in the central nervous system. Oligodendroglia may be called interfascicular, perivascular, or perineuronal (not the same as SATELLITE CELLS, PERINEURONAL of GANGLIA) according to their location. They form the insulating MYELIN SHEATH of axons in the central nervous system.
A progressive form of dementia characterized by the global loss of language abilities and initial preservation of other cognitive functions. Fluent and nonfluent subtypes have been described. Eventually a pattern of global cognitive dysfunction, similar to ALZHEIMER DISEASE, emerges. Pathologically, there are no Alzheimer or PICK DISEASE like changes, however, spongiform changes of cortical layers II and III are present in the TEMPORAL LOBE and FRONTAL LOBE. (From Brain 1998 Jan;121(Pt 1):115-26)
A heterogeneous group of primarily familial disorders characterized by myoclonic seizures, tonic-clonic seizures, ataxia, progressive intellectual deterioration, and neuronal degeneration. These include LAFORA DISEASE; MERRF SYNDROME; NEURONAL CEROID-LIPOFUSCINOSIS; sialidosis (see MUCOLIPIDOSES), and UNVERRICHT-LUNDBORG SYNDROME.
A metallic element with atomic symbol Fe, atomic number 26, and atomic weight 55.85. It is an essential constituent of HEMOGLOBINS; CYTOCHROMES; and IRON-BINDING PROTEINS. It plays a role in cellular redox reactions and in the transport of OXYGEN.
Mice which carry mutant genes for neurologic defects or abnormalities.
The biochemical and electrophysiological interactions between the NERVOUS SYSTEM and IMMUNE SYSTEM.
Compounds or agents that combine with an enzyme in such a manner as to prevent the normal substrate-enzyme combination and the catalytic reaction.
A class of MOLECULAR CHAPERONES found in both prokaryotes and in several compartments of eukaryotic cells. These proteins can interact with polypeptides during a variety of assembly processes in such a way as to prevent the formation of nonfunctional structures.
A general term indicating inflammation of a peripheral or cranial nerve. Clinical manifestation may include PAIN; PARESTHESIAS; PARESIS; or HYPESTHESIA.
The uptake of naked or purified DNA by CELLS, usually meaning the process as it occurs in eukaryotic cells. It is analogous to bacterial transformation (TRANSFORMATION, BACTERIAL) and both are routinely employed in GENE TRANSFER TECHNIQUES.
The entire nerve apparatus, composed of a central part, the brain and spinal cord, and a peripheral part, the cranial and spinal nerves, autonomic ganglia, and plexuses. (Stedman, 26th ed)

Activated human T cells, B cells, and monocytes produce brain-derived neurotrophic factor in vitro and in inflammatory brain lesions: a neuroprotective role of inflammation? (1/2534)

Brain-derived neurotrophic factor (BDNF) has potent effects on neuronal survival and plasticity during development and after injury. In the nervous system, neurons are considered the major cellular source of BDNF. We demonstrate here that in addition, activated human T cells, B cells, and monocytes secrete bioactive BDNF in vitro. Notably, in T helper (Th)1- and Th2-type CD4(+) T cell lines specific for myelin autoantigens such as myelin basic protein or myelin oligodendrocyte glycoprotein, BDNF production is increased upon antigen stimulation. The BDNF secreted by immune cells is bioactive, as it supports neuronal survival in vitro. Using anti-BDNF monoclonal antibody and polyclonal antiserum, BDNF immunoreactivity is demonstrable in inflammatory infiltrates in the brain of patients with acute disseminated encephalitis and multiple sclerosis. The results raise the possibility that in the nervous system, inflammatory infiltrates have a neuroprotective effect, which may limit the success of nonselective immunotherapies.  (+info)

A new X linked neurodegenerative syndrome with mental retardation, blindness, convulsions, spasticity, mild hypomyelination, and early death maps to the pericentromeric region. (2/2534)

We report on a family with an X linked neurodegenerative disorder consisting of mental retardation, blindness, convulsions, spasticity, and early death. Neuropathological examination showed mild hypomyelination. By linkage analysis, the underlying genetic defect could be assigned to the pericentromeric region of the X chromosome with a maximum lod score of 3.30 at theta=0.0 for the DXS1204 locus with DXS337 and PGK1P1 as flanking markers.  (+info)

Increased neurodegeneration during ageing in mice lacking high-affinity nicotine receptors. (3/2534)

We have examined neuroanatomical, biochemical and endocrine parameters and spatial learning in mice lacking the beta2 subunit of the nicotinic acetylcholine receptor (nAChR) during ageing. Aged beta2(-/-) mutant mice showed region-specific alterations in cortical regions, including neocortical hypotrophy, loss of hippocampal pyramidal neurons, astro- and microgliosis and elevation of serum corticosterone levels. Whereas adult mutant and control animals performed well in the Morris maze, 22- to 24-month-old beta2(-/-) mice were significantly impaired in spatial learning. These data show that beta2 subunit-containing nAChRs can contribute to both neuronal survival and maintenance of cognitive performance during ageing. beta2(-/-) mice may thus serve as one possible animal model for some of the cognitive deficits and degenerative processes which take place during physiological ageing and in Alzheimer's disease, particularly those associated with dysfunction of the cholinergic system.  (+info)

Mitochondria in organismal aging and degeneration. (4/2534)

Several lines of experimentation support the view that the genetic, biochemical and bioenergetic functions of somatic mitochondria deteriorate during normal aging. Deletion mutations of the mitochondrial genome accumulate exponentially with age in nerve and muscle tissue of humans and multiple other species. In muscle, a tissue that undergoes age-related fiber loss and atrophy in humans, there is an exponential rise in the number of cytochrome-oxidase-deficient fibers, which is first detectable in the fourth decile of age. Most biochemical studies of animal mitochondrial activity indicate a decline in electron transport activity with age, as well as decreased bioenergetic capacity with age, as measured by mitochondrial membrane potential. Mitochondrial mutations may be both the result of mitochondrial oxidative stress, and cells bearing pure populations of pathogenic mitochondrial mutations are sensitized to oxidant stress. Oxidant stress to mitochondria is known to induce the mitochondrial permeability transition, which has recently been implicated in the release of cytochrome c and the initiation of apoptosis. Thus several lines of evidence support a contribution of mitochondrial dysfunction to the phenotypic changes associated with aging.  (+info)

Apoptosis in neurodegenerative diseases: the role of mitochondria. (5/2534)

Nerve cell death is the central feature of the human neurodegenerative diseases. It has long been thought that nerve cell death in these disorders occurs by way of necrosis, a process characterized by massive transmembrane ion currents, compromise of mitochondrial ATP production, and the formation of high levels of reactive oxygen species combining to induce rapid disruption of organelles, cell swelling, and plasma membrane rupture with a secondary inflammatory response. Nuclear DNA is relatively preserved. Recent evidence now indicates that the process of apoptosis rather than necrosis primarily contributes to nerve cell death in neurodegeneration. This has opened up new avenues for understanding the pathogenesis of neurodegeneration and may lead to new and more effective therapeutic approaches to these diseases.  (+info)

Nitric oxide, mitochondria and neurological disease. (6/2534)

Damage to the mitochondrial electron transport chain has been suggested to be an important factor in the pathogenesis of a range of neurological disorders, such as Parkinson's disease, Alzheimer's disease, multiple sclerosis, stroke and amyotrophic lateral sclerosis. There is also a growing body of evidence to implicate excessive or inappropriate generation of nitric oxide (NO) in these disorders. It is now well documented that NO and its toxic metabolite, peroxynitrite (ONOO-), can inhibit components of the mitochondrial respiratory chain leading, if damage is severe enough, to a cellular energy deficiency state. Within the brain, the susceptibility of different brain cell types to NO and ONOO- exposure may be dependent on factors such as the intracellular reduced glutathione (GSH) concentration and an ability to increase glycolytic flux in the face of mitochondrial damage. Thus neurones, in contrast to astrocytes, appear particularly vulnerable to the action of these molecules. Following cytokine exposure, astrocytes can increase NO generation, due to de novo synthesis of the inducible form of nitric oxide synthase (NOS). Whilst the NO/ONOO- so formed may not affect astrocyte survival, these molecules may diffuse out to cause mitochondrial damage, and possibly cell death, to other cells, such as neurones, in close proximity. Evidence is now available to support this scenario for neurological disorders, such as multiple sclerosis. In other conditions, such as ischaemia, increased availability of glutamate may lead to an activation of a calcium-dependent nitric oxide synthase associated with neurones. Such increased/inappropriate NO formation may contribute to energy depletion and neuronal cell death. The evidence available for NO/ONOO--mediated mitochondrial damage in various neurological disorders is considered and potential therapeutic strategies are proposed.  (+info)

Studies on the role of dopamine in the degeneration of 5-HT nerve endings in the brain of Dark Agouti rats following 3,4-methylenedioxymethamphetamine (MDMA or 'ecstasy') administration. (7/2534)

1. We investigated whether dopamine plays a role in the neurodegeneration of 5-hydroxytryptamine (5-HT) nerve endings occurring in Dark Agouti rat brain after 3,4-methylenedioxymethamphetamine (MDMA or 'ecstasy') administration. 2. Haloperidol (2 mg kg(-1) i.p.) injected 5 min prior and 55 min post MDMA (15 mg kg(-1) i.p.) abolished the acute MDMA-induced hyperthermia and attenuated the neurotoxic loss of 5-HT 7 days later. When the rectal temperature of MDMA + haloperidol treated rats was kept elevated, this protective effect was marginal. 3. MDMA (15 mg kg(-1)) increased the dopamine concentration in the dialysate from a striatal microdialysis probe by 800%. L-DOPA (25 mg kg(-1) i.p., plus benserazide, 6.25 mg kg(-1) i.p.) injected 2 h after MDMA (15 mg kg(-1)) enhanced the increase in dopamine in the dialysate, but subsequent neurodegeneration was unaltered. L-DOPA (25 mg kg(-1)) injected before a sub-toxic dose of MDMA (5 mg kg(-1)) failed to induce neurodegeneration. 4. The MDMA-induced increase in free radical formation in the hippocampus (indicated by increased 2,3- and 2,5-dihydroxybenzoic acid in a microdialysis probe perfused with salicylic acid) was unaltered by L-DOPA. 5. The neuroprotective drug clomethiazole (50 mg kg(-1) i.p.) did not influence the MDMA-induced increase in extracellular dopamine. 6. These data suggest that previous observations on the protective effect of haloperidol and potentiating effect of L-DOPA on MDMA-induced neurodegeneration may have resulted from effects on MDMA-induced hyperthermia. 7. The increased extracellular dopamine concentration following MDMA may result from effects of MDMA on dopamine re-uptake, monoamine oxidase and 5-HT release rather than an 'amphetamine-like' action on dopamine release, thus explaining why the drug does not induce degeneration of dopamine nerve endings.  (+info)

Possible role of NF-kappaB and p53 in the glutamate-induced pro-apoptotic neuronal pathway. (8/2534)

Apoptosis is now recognized as an important component in many progressive and acute neurodegenerative diseases. Extracellular signals and intracellular mechanisms triggering and regulating apoptosis in neuronal cells are still a matter of investigation. Here we review data from our and other laboratories with the aim to elucidate the nature of some proteins which are known to be involved in cell cycle regulation as well as in promoting degeneration and apoptosis of neurons. The following molecules will be taken into consideration: NF-kappaB, p53, p21 and MSH2. These proteins are activated by neurotoxic experimental conditions which involve the stimulation of selective receptors for the excitatory aminoacid glutamate. Thus, we hypothesize their contribution to an intracellular pathway responsible for the glutamate-induced neuronal death. Identification of such mechanisms could be relevant for understanding the apoptosis associated with various neurodegenerative diseases as well as for developing novel strategies of pharmacological intervention.  (+info)

The EU Joint Programme - Neurodegenerative Disease Research (JPND) initiative launched a new joint transnational call for multinational research projects on personalised medicine for neurodegenerative diseases. The call is launched in partnership with the European Commission and has a budget of ca. EUR 30 M.. Neurodegenerative diseases are debilitating and still largely untreatable conditions. They are characterised by a large variability in their origins, mechanisms and clinical expression. When searching for a medical solution, e.g. a treatment or an optimised approach for care, this large variability constitutes a major hurdle if not controlled. Indeed a treatment addressing one disease pathway may not be useful for all patients experiencing the relevant symptoms. Thus, one of the greatest challenges for treating neurodegenerative diseases is the deciphering of this variability.. The following neurodegenerative diseases are included in the call:. ...
Neitzel, J.; Watts, M.; Diehl-Schmid, J.; Ortner, M.; Grimmer, T.; Yakushev, I.; Bublak, P.; Preul, C.; Finke, K.; Sorg, C. (2016): Distinct subcortical atrophy patterns across four different neurodegenerative syndromes. In: Journal of Neurochemistry, Vol. 138: p. 385 ...
The human APOE gene, which codes for apolipoprotein E (apoE), has three major polymorphic alleles: ε2, ε3, and ε4 that give rise to amino acid substitutions. APOE-ε4 is a strong risk factor of sporadic Alzheimers disease (AD) but the reason why is still unknown despite intense research for more than 20 years. The aim of the study was to investigate if the concentrations of total apoE and the specific apoE isoforms in cerebrospinal fluid (CSF) differ between various neurodegenerative diseases and control individuals, as well as among the APOE genotypes. Quantification of total apoE and specific apoE isoforms (E2, E3, and E4) in CSF was performed using high-resolution parallel reaction monitoring mass spectrometry. In total, 1820 individuals were involved in the study including clinically diagnosed AD patients (n = 228), cognitively unimpaired (CU) patients (n = 896), and patients with other neurodegenerative disorders (n = 696). Follow-up data was available for 100 individuals, assessed at two time
Aggregation of misfolded proteins and the associated loss of neurons are considered as a hallmark of numerous neurodegenerative diseases. Optineurin is present in protein inclusions observed in various neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), Huntingtons disease, Alzheimers disease, Parkinsons disease, Creutzfeld-Jacob disease and Picks disease. Optineurin deletion mutations have also been described in ALS patients. However, the role of optineurin in mechanisms of protein aggregation remains unclear. In this report, we demonstrate that optineurin recognized various protein aggregates via its C-terminal coiled-coil domain in a ubiquitin-independent manner. We also show that optineurin depletion significantly increase protein aggregation in HeLa cells and morpholino-silencing of the optineurin ortholog in zebrafish causes the motor axonopathy phenotype similar to a zebrafish model of ALS. A more severe phenotype is observed when optineurin is depleted in ...
Abstract Platelets play an important role in a variety of disorders viz; cardiovascular, psychosomatic, psychiatric, thrombosis, HIV/AIDS in addition to various neurodegenerative diseases (NDD). Recent evidence indicates that platelet react to divers
BACKGROUND & OBJECTIVE: Alzheimers disease (AD) and Parkinsons disease (PD) affect an increasing number of the elderly population worldwide. The existing treatments mainly improve the core symptoms of AD and PD in a temporary manner and cause alarming side effects. Naturally occurring flavonoids are well-documented for neuroprotective and neurorestorative effects against various neurodegenerative diseases. Thus, we analyzed the pharmacokinetics of eight potent natural products flavonoids for the druggability and discussed the neuroprotective and neurorestorative effects and the underlying mechanisms. CONCLUSION: This review provides valuable clues for the development of novel therapeutics against neurodegenerative diseases ...
Neurodegenerative diseases are among the most common causes of disability worldwide. Although neurodegenerative diseases are heterogeneous in both their clinical features and the underlying physiology, they are all characterised by progressive loss of specific neuronal populations. Recent experiment …
This book sheds new light on neurodegenerative disorders as systemic diseases. Classically, neuronal cell death was a hallmark of such disorders. However, it has become evident that neural dysfunction is more important in the pathophysiology of neurodegenerative disorders. More recently, the prionoid-spreading hypothesis of disease-causing molecules has attracted a great deal of attention. Therapeutic strategies thus must be reconsidered in the light that neurodegenerative disorders are indeed systemic diseases. The first part of this book introduces the concept of neurodegeneration in biology and pathophysiology. The second part focuses on clinical evaluation and biomarkers from the perspective of this new concept, while the third summarizes the risk factors of neurodegeneration. The fourth part of this work indicates future directions of treatment, and the final part discusses health promotion for prevention and quality of life. This book will be of interest to both researchers and medical ...
Neurodegenerative diseases affect millions of people worldwide, and Alzheimers disease and Parkinsons disease are the most common types. The risk of being affected by a neurodegenerative disease increases dramatically with age and despite substantial research and development investments, effective therapeutics for the millions of patients with neurodegenerative diseases remain elusive. This creates a critical need to improve our understanding of what causes neurodegenerative diseases and develop new approaches for treatment and prevention.. USC is uniquely positioned as one of the top research institutions in the nation with comprehensive neurodegenerative disease research capabilities from bench to bedside and back. USC is comprised of leading scientists contributing to better understanding of the pathogenesis and treatment of neurological disorders such as Alzheimers disease and stroke, and leading experts in big data neuro-informatics, neuroimaging, structure-based drug discovery, systems ...
Neurodegenerative diseases affect millions of people worldwide, and Alzheimers disease and Parkinsons disease are the most common types. More than five million Americans are living with Alzheimers disease, and at least 500,000 Americans live with Parkinsons disease, although some estimates are much higher.. Neurodegenerative diseases occur when nerve cells in the brain or peripheral nervous system lose function over time and ultimately die. Although treatments may help relieve some of the physical or mental symptoms associated with neurodegenerative diseases, there is currently no cure or way to slow disease progression.. The risk of being affected by a neurodegenerative disease increases dramatically with age. Population-wide health improvements have increased lifespan, which along with a larger generation of aging Americans means more people may be affected by neurodegenerative diseases in coming decades. This creates a critical need to improve our understanding of what causes ...
Living cells continually generate reactive oxygen species (ROS) through the respiratory chain during energetic metabolism. ROS at low or moderate concentration can play important physiological roles. However, an excessive amount of ROS under oxidative stress would be extremely deleterious. The central nervous system (CNS) is particularly vulnerable to oxidative stress due to its high oxygen consumption, weakly antioxidative systems and the terminal-differentiation characteristic of neurons. Thus, oxidative stress elicits various neurodegenerative diseases. In addition, chemotherapy could result in severe side effects on the CNS and peripheral nervous system (PNS) of cancer patients, and a growing body of evidence demonstrates the involvement of ROS in drug-induced neurotoxicities as well. Therefore, development of antioxidants as neuroprotective drugs is a potentially beneficial strategy for clinical therapy. In this review, we summarize the source, balance maintenance and physiologic functions of ROS,
Functional and structural connectivity measures, as assessed by means of functional and diffusion MRI, are emerging as potential intermediate biomarkers for Alzheimer disease (AD) and other disorders. This Review aims to summarize current evidence that connectivity biomarkers are associated with upstream and downstream disease processes (molecular pathology and clinical symptoms, respectively) in the major neurodegenerative diseases. The vast majority of studies have addressed functional and structural connectivity correlates of clinical phenotypes, confirming the predictable correlation with topography and disease severity in AD and frontotemporal dementia. In neurodegenerative diseases with motor symptoms, structural--but, to date, not functional--connectivity has been consistently found to be associated with clinical phenotype and disease severity. In the latest studies, the focus has moved towards the investigation of connectivity correlates of molecular pathology. Studies in cognitively healthy
ApoE is the main lipid-carrier protein in the brain and has a role in the transport and metabolism of many lipid classes. This is important because the brain is a fatty, lipid-rich organ. ApoE can take three different forms, and the consequences of these differences can profoundly affect processes like lipid homeostasis and neurodegeneration. One specific form, ApoE4, increases ones risk of developing Alzheimers by as much as eightfold. A recent thematic review series in the Journal of Lipid Research includes eight articles that explore the connections between brain lipids, ApoE and Alzheimers disease.. Lipid abnormalities are closely associated with various neurodegenerative diseases, said Ta-Yuan T.Y. Chang of Geisel School of Medicine at Dartmouth College. He and wife Catherine Chang have been researching cholesterol metabolism and its relationship to neurodegenerative diseases for more than four decades. As the coordinators of the series, they selected experts in their fields to ...
In a large multi-site study, the researchers identified more than 1,500 potential biomarkers in cerebrospinal fluid from patients with one of three neurodegenerative diseases: Alzheimers, Parksinsons, or dementia with Lewy bodies (DLB). Researchers identified different sets of potential biomarkers corresponding to each disease; each of the proteins are linked specifically to one of the diseases. The results appear in the new issue of the Journal of Alzheimers Disease.. Were getting very close to being able to use these biomarkers for the clinical diagnosis of Alzheimers and Parkinsons disease, and dementia with Lewy bodies, said the studys lead author, Dr. Jing Zhang, associate professor of pathology at the UW. His lab is at Harborview Medical Center. This is a major improvement on other biomarker detection techniques.. Alzheimers, Parkinsons, and other neurodegenerative diseases affect millions of people in the United States, and the toll of the diseases is expected to worsen as ...
Defects in kinesin-3 transport have been implicated in diverse genetic, developmental, neurodegenerative and cancer diseases. Despite their widespread functions and clinical importance, the mechanisms of kinesin-3 mediated intracellular transport, regulations and their deficiencies in the context of human diseases are largely unknown. The project will continue to investigating the members of this family at cellular, molecular, structural and single molecule level to gain fundamental insights into molecular mechanisms of neuronal transport systems and the implications for various neurodegenerative diseases caused by the defects in microtubule based transport system. We will use cultured hippocampal neurons and Caenorhabditis elegans as model systems ...
This programme of research is aimed at studying underlying mechanisms and treatments for the major neurodegenerative diseases - Alzheimers, Epilepsy, Parkinsons, and Huntingtons. Neurodegenerative diseases affect over 100,000 New Zealanders and this will markedly increase as the population ages.
The new research suggests that the cells may die because of naturally occurring gene variation in brain cells that were, until recently, assumed to be genetically identical. This variation - called somatic mosaicism - could explain why neurons in the temporal lobe are the first to die in Alzheimers, for example, and why dopaminergic neurons are the first to die in Parkinsons.. This has been a big open question in neuroscience, particularly in various neurodegenerative diseases, said neuroscientist Michael McConnell, PhD, of UVAs Center for Brain Immunology and Glia (BIG). What is this selective vulnerability? What underlies it? And so now, with our work, the hypotheses moving forward are that it could be that different regions of the brain actually have a different garden of these [variations] in young individuals and that sets up different regions for decline later in life.. A Most Unexpected Outcome. The finding emerged unexpectedly from McConnells investigations into schizophrenia. ...
This section examines evidence for neurovascular changes during normal ageing and for neurovascular and/or BBB dysfunction in various neurodegenerative diseases, as well as the possibility that vascular defects can precede neuronal changes.. Age-associated neurovascular changes. Normal ageing diminishes brain circulatory functions, including a detectable decay of CBF in the limbic and association cortices that has been suggested to underlie age-related cognitive changes77. Alterations in the cerebral microvasculature, but not changes in neural activity, have been shown to lead to age-dependent reductions in functional hyperaemia in the visual system in cats78 and in the sensorimotor cortex in pericyte-deficient mice33. Importantly, a recent longitudinal CBF study in neurologically normal individuals revealed that people bearing the apolipoprotein E (APOE) ɛ4allele - the major genetic risk factor for late-onset Alzheimers disease79, 80, 81 - showed greater regional CBF decline in brain regions ...
p,Striatal interneurons display a morphological and chemical heterogeneity that has been particularly well characterized in rats, monkeys and humans. By comparison much less is known of striatal interneurons in mice, although these animals are now widely used as transgenic models of various neurodegenerative diseases. The present immunohistochemical study aimed at characterizing striatal interneurons expressing calretinin (CR) in mice compared to those in squirrel monkeys and humans. The mouse striatum contains both small (9-12 μm) and medium-sized (15-20 μm) CR+ cells. The small cells are intensely stained with a single, slightly varicose and moderately arborized process. They occur throughout the striatum (77±9 cells/mm(3)), but prevail in the area of the subventricular zone and subcallosal streak, with statistically significant anteroposterior and dorsoventral decreasing gradients. The medium-sized cells are less intensely immunoreactive and possess 2-3 long, slightly varicose and poorly ...
Thanks in large part to the seminal work of Steve White and his colleagues, we appreciate the ordered complexity of the lipid bilayer and how it impacts the incorporation of integral membrane proteins as well as more peripherally associated proteins. Steves work also provides a vital foundation to tackle another challenge: cytotoxic oligomeric complexes which accumulate in various neurodegenerative diseases. These oligomers have a relatively fluid structure and interact with many different proteins in the cell, but their main target is thought to be the phospholipid membrane, either the plasma membrane or internal organelles such as the mitochondria. This fascinating encounter between two essentially fluid phases generates a more disordered membrane, and presumably promotes uncontrolled transport of small metal ions across the membrane barrier. Happily, this unwanted interaction may be suppressed by mobilizing the phospholipid bilayer into its own defense. Extruded nanolipoparticles (NLPs) ...
Title: Cannabinoids and Neurodegenerative Diseases. VOLUME: 8 ISSUE: 6. Author(s):Julian Romero and Jose Martinez-Orgado. Affiliation:Laboratorio de Investigacion, Hospital Universitario Fundacion Alcorcon and Centro de Investigacion Biomedica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), C/o Budapest 1, 28922, Alcorcon, Spain.. Keywords:Alzheimers disease, ischemia, neuroprotection, glia. Abstract: Although significant advances have taken place in recent years on our understanding of the molecular mechanisms of different neurodegenerative diseases, its translation into effective therapeutic treatments has not been as successful as could be expected. There is still a dramatic lack of curative treatments for the most frequent disorders and only symptomatic relief for many others. Under this perspective, the search for novel therapeutic approaches is demanding and significant attention and efforts have been directed to studying additional neurotransmission systems including the ...
Lou Gehrigs disease, known as amyotrophic lateral sclerosis or ALS, strikes healthy, middle-aged people seemingly at random. Of the major neurodegenerative diseases, it has the least hope for treatment and survival. Although mental capabilities stay intact, ALS paralyzes people,....... ...
Neurological disorders have a strong negative impact on the quality of life of affected patients, their close relatives as well as on the health economic system. Early diagnosis associated with better treatment outcomes remain difficult to reach. Similarly, misdiagnosis is frequent and can lead to the delivery of the wrong treatment to the patient. As a matter of fact, the therapeutic strategies developed to treat Alzheimers Disease (AD) and Parkinsons Disease (PD), two major neurodegenerative diseases, are unfortunately only effective for reducing the symptoms. This illustrates the lack of complete understanding of the mechanisms underlining neurodegeneration. In this context, this thesis aims to contribute to a precise comprehension of the synaptic transmission, at the cellular level, by providing a biocompatible brain interface capable of collecting neurochemicals while establishing a tight electrical connection with the cerebral tissues. The first part of this thesis deals with the design ...
Lou Gehrigs disease, known as amyotrophic lateral sclerosis or ALS, strikes healthy, middle-aged people seemingly at random. Of the major neurodegenerative diseases, it has the least hope for treatment and survival. Although mental capabilities stay intact, ALS paralyzes people,....... ...
DESCRIPTION (provided by applicant): Methylation is a ubiquitous covalent modification used to control the function of diverse biomolecules including hormones, neurotransmitters, xenobiotics, proteins, nucleic acids and lipids. More than 50 distinct methyltransferase (MTs) enzymes are present in humans, and they are being targeted for a broad range of diseases, but their involvement in neurodegenerative disease pathways is of special relevance. Modulation of neurotransmitter methylation is an emerging therapeutic strategy for the treatment of several neurodegenerative diseases, most notably Parkinsons and Alzheimers diseases, and DNA and protein MTs are also being targeted for neurodegenerative diseases and cancers of the CNS. Moreover, the involvement of some MT family members in disease pathways is intertwined with their role in metabolizing commonly prescribed drugs. The development of highly selective MT modulators is clearly a compelling medical priority. However, efforts to achieve this ...
An expert panel convened by the NIH found little evidence that Alzheimers, Parkinsons, and similar neurodegenerative diseases can be transmitted in a prion-like fashion, such as after contact with biological specimens or contaminated surgical instruments. Still, writing in the October Journal of Neuropathology and Experimental Neurology, the panel, led by Matthew Frosch, Massachusetts General Hospital, Boston, consider current research on this topic inadequate and recommend that critical questions be studied so any risk of iatrogenic spread can be truly assessed. The panel broadly agrees with a whitepaper a European working group recently published on the topic (Sep 2020 news). Toxic forms of amyloid-β, tau, and α-synuclein can act as seeds to coax misfolding and aggregation of normal versions of these proteins in healthy brains. For example, injecting minuscule amounts of toxic Aβ into mouse brain prompts rampant formation of amyloid plaques months later (Oct 2011 news). Researchers have ...
Neurodegenerative diseases are characterized by the loss of nerve cells. It is generally accepted that once the cells of the brain and spinal cord are damaged they are not easily regenerated. The three most common neurodegenerative diseases are Alzheimers disease, Parkinsons disease and Multiple Sclerosis.
A new firm, Yumanity therapeutics, has been launched with the goal of working on neurodegenerative diseases. Presently almost 50 million people worldwide suffer from multiple types of neurodegenerative disease. News on NewsHub.org
Abstract Neurodegenerative diseases are a heterogeneous, mostly age-associated group of disorders characterized by progressive neuronal loss, the most prevalent being Alzheimer disease. It is anticipated that, with continuously increasing life expectancy, these diseases will pose a serious social and health problem in the near feature. Meanwhile, however, their etiology remains largely obscure even though all possible novel clues are being thoroughly examined. In this regard, a concept has been proposed that p53, as a transcription factor controlling many vital cellular pathways including apoptosis, may contribute to neuronal death common to all neurodegenerative disorders. In this work, we review the research devoted to the possible role of p53 in the pathogenesis of these diseases. We not only describe aberrant changes in p53 level/activity observed in CNS regions affected by particular diseases but, most importantly, put special attention to the complicated reciprocal regulatory ties existing ...
TY - JOUR. T1 - Cytokine/neurotrophin interaction in the aged central nervous system. AU - Macdonald, Nancy J.. AU - Decorti, Francesco. AU - Pappas, Todd C.. AU - Taglialatela, Giulio. PY - 2000. Y1 - 2000. N2 - Age-associated neurodegenerative diseases such as Alzheimers disease are characterised by neuronal impairment that leads to cognitive deficits. As certain affected neurons depend on trophic factors such as neurotrophins (NTs), impairment in NT function has been suggested to be a component of neuronal damage associated with such disorders. Age-related neurodegenerative diseases are also characterised by high levels of proinflammatory cytokines such as tumour necrosis factor alpha (TNFα) in the CNS. Because TNFα receptors and certain NT receptors share a high degree of homology and are capable of activating similar signalling pathways, one possibility is that altered cytokine levels may affect NT function in the aged or diseased CNS. Here we wish briefly to review the evidence ...
SingHealth Foundation Grant. Overview. Our laboratorys interests lie in identification and verification of novel key molecular targets, signaling pathways or neuro-protective agents relevant to pathogenesis and therapy of debilitating human neurodegenerative diseases, including Alzheimers disease (AD), Parkinson disease (PD) and multiple sclerosis. To achieve it, cutting-edged high throughput screening works are being performed in the lab. First, the in vitro high throughput proteomics screening plus immuno-precipitation protocols as well as cellular and molecular techniques are utilized to search and identify new key molecular targets or novel signaling pathways relevant to pathogenesis and therapy in human neurodegenerative diseases. Second, in vitro high throughput chemical library screening is being performed to identify new neuro-protective agents to modulate identified new molecular targets or signaling pathways for future clinical drugs developments. Findings from in vitro ...
Neuronal cell loss contributes to the pathology of acute and chronic neurodegenerative diseases, including Alzheimers disease (AD). It remains crucial to identify molecular mechanisms sensitizing neurons to various insults and cell death. To date, the multifunctional, autophagy-related protein Beclin 1 has been shown to be both necessary and sufficient for neuronal integrity in neurodegenerative models associated with protein aggregation. Interestingly, besides its role in cellular homeostasis, Beclin 1 has also been ascribed a role in apoptosis. This makes it critical to elucidate whether Beclin 1 regulates neuronal death and survival across neurodegenerative conditions independent of protein clearance. Here, we provide experimental evidence for a direct functional link between proteolytic cleavage of Beclin 1 and apoptotic neuronal cell loss in two independent models of neurodegeneration in vivo. Proteolytic cleavage of Beclin 1 was characterized in lysates of human AD brain samples. We developed
Neurodegenerative diseases, such as Alzheimers disease (AD), Parkinsons disease (PD), amyotrophic lateral sclerosis (ALS) and Huntingtons disease (HD) are caused by the progressive loss of neuronal integrity or acute neuron injury such as stroke or trauma in the brain and spinal cord.1-3 Current therapeutic strategies for neurodegenerative diseases are mainly aimed to decrease CNS neuron damage or brain dysfunction by conferring neuroprotection and neurogenesis.4,5 Consequently, the development of effective therapeutic medicine or discovery of new biological targets with neurogenesis activities remains an urgent need in the treatment of neurodegenerative diseases. During the past decades, various kinds of receptors, ion channels and signaling pathways associated with neurogenesis activities have been identified as potential therapeutic targets for neurodegenerative diseases. Among these, the σ1 receptor has attracted wide attention.6 σ1 receptor is one of the subtype belonging to the σ ...
Many of the key findings in neurodegenerative disease are from MIND. SOD1, the most common mutation associated with ALS was identified at MIND. MIND researchers had a critical role in identifying every one of the Alzheimers genes that has been found except for ApoE. Additionally, the gene for Huntingtons disease, which has paved the way for greater understanding of the disease, was discovered at Mass General.. ...
Many neurodegenerative diseases are characterized by the conformational change of self-proteins into amyloidogenic, pathological conformers, which share structu...
Our research focuses on identifying pathophysiological consequences of widespread RNA dysregulation in neurodegeneration in order to design, develop and test novel strategies of neuroprotective therapies.. Widespread dysregulation of the RNA metabolism has been recognised as a key pathophysiological component causing at least four neurodegenerative disorders: motor neurone disease (MND), also called Amyotrophic Lateral Sclerosis (ALS), spinal muscular atrophy (SMA), Huntingtons disease (HD) and spinocerebellar ataxias (SCAs). Widespread alteration of the transcriptome has also been reported in normal ageing of the brain and many neurodegenerative disorders are late progressive adult-onset diseases. Neurodegeneration in Parkinsons disease (PD) or Alzheimers disease (AD) is also likely to exhibit and/or involve broad alteration of the RNA metabolism and of multiple biological processes.. Although some genetic causes of these often-fatal diseases are known, the multifactorial molecular ...
A team of researchers, led by scientists at the University of California, San Diego, have identified a key player in the dramatic loss of neurons in mice and fly models, a discovery that could help illuminate the role of mitochondrial dysfunction in human neurodegenerative disorders, such as Parkinsons disease.
As a model organism |i|Saccharomyces cerevisiae|/i| has greatly contributed to our understanding of many fundamental aspects of cellular biology in higher eukaryotes. More recently, engineered yeast models developed to study endogenous or heterologous proteins that lay at the root of a given disease have become powerful tools for unraveling the molecular basis of complex human diseases like neurodegeneration. Additionally, with the possibility of performing target-directed large-scale screenings, yeast models have emerged as promising first-line approaches in the discovery process of novel therapeutic opportunities against these pathologies. In this paper, several yeast models that have contributed to the uncovering of the etiology and pathogenesis of several neurodegenerative diseases are described, including the most common forms of neurodegeneration worldwide, Alzheimers, Parkinsons, and Huntingtons diseases. Moreover, the potential input of these cell systems in the development of more
During embryonic development, sensory and motor fibers interact to form nerves in the limbs. The research team led by Dr. Andrea Huber Brösamle of the Institute of Developmental Genetics of Helmholtz Zentrum München has now elucidated how this interaction functions at the molecular level: The cell surface receptor neuropilin-1 is present in both sensory and motor nerve fibers and controls their interaction in order to correctly regulate growth. We observed that motor and sensory axons were both able to guide and lead the formation of the spinal nerves of the arms and legs, said Rosa-Eva Hüttl and Heidi Söllner, lead authors of the study and doctoral students in Dr. Andrea Huber Brösamles research group. This finding surprised the authors because it had previously been assumed that the motor axons were always responsible for establishing the correct trajectories. In the same study, the researchers created a model to better elucidate structural changes in human neurodegenerative disorders ...
Peptides that act like small molecules are highly desirable as drug candidates for targeting larger proteins and peptide specific binding sites. Hybridtides are
Ayer AH, Wojta K, Ramos EM, Dokuru D, Chen JA, Karydas AM, Papatriantafyllou JD, Agiomyrgiannakis D, Kamtsadeli V, Tsinia N, Sali D, Gylys KH, Agosta F, Filippi M, Small GW, Bennett DA, Gearing M, Juncos JL, Kramer J, Lee SE, Yokoyama JS, Mendez MF, Chui H, Zarow C, Ringman JM, Kilic U, Babacan-Yildiz G, Levey A, DeCarli CS, Cotman CW, Boxer AL, Miller BL, Coppola G. Frequency of the TREM2 R47H Variant in Various Neurodegenerative Disorders.. Alzheimer disease and associated disorders, 2019 ...
Biometals in Neurodegenerative Diseases: Mechanisms and Therapeutics is an authoritative and timely resource bringing together the major findings in the field for ease of access to those working in the field or with an interest in metals and their role in brain function, disease, and as therapeutic targets. Chapters cover metals in Alzheimers Disease, Parkinsons Disease, Motor Neuron Disease, Autism and lysosomal storage disorders.. This book is written for academic researchers, clinicians and advanced graduate students studying or treating patients in neurodegeneration, neurochemistry, neurology and neurotoxicology. The scientific literature in this field is advancing rapidly, with approximately 300 publications per year adding to our knowledge of how biometals contribute to neurodegenerative diseases.. Despite this rapid increase in our understanding of biometals in brain disease, the fields of biomedicine and neuroscience have often overlooked this information. The need to bring the ...
Many questions remain about how and when CCSVI might play a role in nervous system damage and whether venous angioplasty is helpful in treating the symptoms of CCSVI. Utilizing intravascular ultrasound (IVUS,) this pilot study will provide data that will allow researchers to evaluate venous morphology pre- and post- percutaneous angioplasty and sub-classify valve morphology as related to treatment success by distinguishing vessels which are more responsive to treatment. In addition, this study will validate the safety of valvuloplasty in various neurodegenerative disorders that involve venous obstruction ...
Abnormal proteostasis due to alterations in protein turnover has been postulated to play a central role in several neurodegenerative diseases. Therefore, the development of techniques to quantify protein turnover in the brain is critical for understanding the pathogenic mechanisms of these diseases. We have developed a bolus stable isotope-labeling kinetics (SILK) technique coupled with multiple reaction monitoring mass spectrometry to measure the clearance of proteins in the mouse brain. Cohorts of mice were pulse labeled with 13 C6-leucine and the brains were isolated after pre-determined time points. The extent of label incorporation was measured over time using mass spectrometry to measure the ratio of labeled to unlabeled apolipoprotein E (apoE) and amyloid β (Aβ). The fractional clearance rate (FCR) was then calculated by analyzing the time course of disappearance for the labeled protein species. To validate the technique, apoE clearance was measured in mice that overexpress the low-density
This study is being conducted to better understand the role of inflammation in Parkinsons disease (PD) and Alzheimers disease (AD). The investigators plan to recruit 30 PD, 30 AD/Amnestic Mild Cognitive Impairment (aMCI), and 60 age matched healthy controls in this study to study the role of immune response in PD and AD.. The study involves up to two study visits involving brief questionnaires and blood draw of up to 250cc (approximately 17 tablespoons) to be collected. More ways to participate, including 1) smaller amount blood donation (up to 100cc per visit for 1-2 visits); and 2) participation via tele-visit and mobile phlebotomy visits (blood donation up to 50cc, ~5 tubes, by a certified mobile phlebotomist at home/location of choice) now available. ...
Neurodegenerative Diseases is a bimonthly, multidisciplinary journal for the publication and discussion of advances in research on all aspects of neurodegenerative diseases. The journal focuses on Alz
Parkinsons disease is a neurodegenerative disorder of unknown cause that particularly affects areas of the brain which are involved in movement control. Research studies confirm the value of motor learning in Parkinsons disease, as well as showing improvements as a result of training.. CuPiD will develop innovative rehabilitation based on new technology, the patients needs, the principles of motor learning in Parkinsons disease. CuPiD will contribute to the challenge of engaging patients with a chronic neurodegenerative disease in intensive exercise for a considerable length of time.. ...
Parkinsons disease is a neurodegenerative disorder of unknown cause that particularly affects areas of the brain which are involved in movement control. Research studies confirm the value of motor learning in Parkinsons disease, as well as showing improvements as a result of training.. CuPiD will develop innovative rehabilitation based on new technology, the patients needs, the principles of motor learning in Parkinsons disease. CuPiD will contribute to the challenge of engaging patients with a chronic neurodegenerative disease in intensive exercise for a considerable length of time.. ...
Proteins, the components of our body that execute, control and organize basically all functions in our cells, are made out of strings of amino acids, which - like an origami - are folded into specific and complex three-dimensional structures according to their desired functions. However, since folding and maintaining of such structures is highly sensitive to cellular or environmental stress, proteins can potentially misfold or form clumps (aggregates). Such undesired protein waste can be toxic for cells and may even lead to cell death. Because several human neurodegenerative diseases are known to be linked to an accumulation of abnormal protein aggregates, basic science aimed to understand how cells remove cellular garbage is elementary for designing strategies for a potential prevention or cure of such disorders. Scientists in the laboratory of Stefan Jentsch at the MPIB now successfully used bakers yeast for screening for new cellular waste disposal pathways. Kefeng Lu, a postdoctoral ...
TY - JOUR. T1 - Quantitative measurement of postural sway in mouse models of human neurodegenerative disease. AU - Hutchinson, D.. AU - Ho, V.. AU - Dodd, M.. AU - Dawson, H. N.. AU - Zumwalt, A. C.. AU - Schmitt, D.. AU - Colton, C. A.. PY - 2007/9/21. Y1 - 2007/9/21. N2 - Detection of motor dysfunction in genetic mouse models of neurodegenerative disease requires reproducible, standardized and sensitive behavioral assays. We have utilized a center of pressure (CoP) assay in mice to quantify postural sway produced by genetic mutations that affect motor control centers of the brain. As a positive control for postural instability, wild type mice were injected with harmaline, a tremorigenic agent, and the average areas of the 95% confidence ellipse, which measures 95% of the CoP trajectory values recorded in a single trial, were measured. Ellipse area significantly increased in mice treated with increasing doses of harmaline and returned to control values after recovery. We also examined postural ...
Series: Neuroscience Research Progress. BISAC: MED057000. Multiple advanced neuroimaging applications in various neurodegenerative diseases including Parkinsons disease (PD), frontotemporal dementia (FTD), vascular dementia (VaD) and autism spectrum disorder (ASD) are covered in this book. Relatively novel techniques such as integrated PET/MRI and independent component analysis (ICA)-based dual regression (DR) methods were developed to capture multi-level molecular/functional and structural/microstructural as well as high-order inter-network coordination abnormalities. For instance, both PET dopamine transporter and striatal binding ratio reductions in the caudate and putamen were found in PD, consistent with the diffusion tensor imaging (DTI) fractional anisotropy (FA) reduction and fMRI voxel-mirrored homotopic correlation (VMHC) in the substantia nigra (swallow tail sign signature of PD). Furthermore, dopamine storage and pathway labeled with the vesicular monoamine transporter tracer ...
Hexanucleotide repeat expansions of variable size in C9orf72 are the most prevalent genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. Sense and antisense transcripts of the expansions are translated by repeat-associated non-AUG translation into five dipeptide repeat proteins (DPRs). Of these, the polyGR, polyPR and, to a lesser extent, polyGA DPRs are neurotoxic, with polyGA the most abundantly detected DPR in patient tissue. Trans-cellular transmission of protein aggregates has recently emerged as a major driver of toxicity in various neurodegenerative diseases. In vitro evidence suggests that the C9 DPRs can spread. However, whether this phenomenon occurs under more complex in vivo conditions remains unexplored. Here, we used the adult fly brain to investigate whether the C9 DPRs can spread in vivo upon expression in a subset of neurons. We found that only polyGA can progressively spread throughout the brain, which accumulates in the shape of aggregate-like puncta inside
Mutations in the Valosin containing protein (VCP) gene are the cause of various neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS), a form of motor neuron disease, and a rare multisystem disease which affects muscle, bone and brain. VCP is involved in a lot of different functions in cells. We know that VCP is important…
Abnormal iron accumulation within the brain is associated with various neurodegenerative diseases; however, there is debate about whether milder disorders of systemic iron loading, such as haemochromatosis, affect the brain. Arguments on both sides of the debate are often based on some common assumptions that have not been rigorously tested by appropriate experimentation. Recent research from our lab has applied high-throughput molecular techniques such as microarray to models of dietary and genetic iron loading to identify subtle but important effects on molecular systems in the brain that may go undetected by other methods commonly used in the field. In this chapter, we review the existing research in animal models and human patients and discuss the strengths and limitations of the different approaches commonly used. Using our findings as an example, we argue that transcriptomic methods can provide unique insights into how systemic iron loading can affect the brain and suggest some basic ...
A link between DNA damage and the process of aging has been firmly established (1, 2). The brain in particular is a vulnerable organ that is plagued by various neurodegenerative disorders that have been related to aging, i.e. Alzheimer and Parkinson disease. The study of the early onset of age-related neurodegenerative diseases is challenging, because there are not many confident early molecular determinants that predict their development. Therefore, progeroid syndromes (showing premature aging) are often used as a model for segmental aging as they show consistent and predictive elements of the aging phenotype (e.g. cessation of growth and development, hearing loss, and severe and progressive neuron dysfunction) (1, 3). These accelerated aging syndromes have in common that they carry defects in one or multiple proteins involved in DNA damage repair mechanisms.. A well established progeroid mouse model is the excision repair cross-complementing group 1 (Ercc1)1 gene knock-out (4, 5). The ...
28 04 2020GABA or gamma-aminobutyric acid is the major inhibitory neurotransmitter in mammals brains GABA receptors are the most common single receptor found in the synapses where neurons communicate with each other There are two known types of GABA receptor: GABA-A which comprise the primary sites of sedative drug action and GABA-B which play a role in muscle tone regulation Abstract Current treatments for insomnia such as zolpidem (Ambien) and eszopiclone (Lunesta) are γ-aminobutyric acid type A (GABA A)-positive allosteric modulators that carry a number of side effects including the potential to disrupt cognition In an effort to develop better tolerated medicines we have identified dual orexin 1 and 2 receptor antagonists (DORAs) which promote sleep in Because the GABA receptor is abundant in the cortex and is very sensitive to damage it represents a reliable marker of neuronal integrity-for example in ischemic brain damage and in various neurodegenerative diseases Part of the GABA A ...
When Dr. Jeff Bradstreet unexpectedly and tragically died in the spring of 2015, many of us wondered what would happen to the research he was doing with GcMAF, Goleic, and Bravo yogurt, which he was using to help children with autism. To my great pleasure, I am pleased to report that the research has continued and treatment options have been expanded. We are now seeing even more powerful results for the treatment of autism, cancer, chronic Lyme, chronic fatigue syndrome, and various neurodegenerative diseases. Dr. Bradstreets previous research focused on the macrophage activating factors known as GcMAF and Goleic, which he used in his clinic. Dr. Bradstreet was able to help 3 out of 4 autistic children by treating them with GcMAF or Goleic. Approximately 20% to 25% of these children lost their autism diagnosis and another 50% experienced a reduction in autistic symptoms. After the European manufacturing facility for GcMAF and Goleic was raided and closed down in the first months of 2015, Dr. ...
Elevated expression of specific transposable elements (TEs) has been observed in several neurodegenerative disorders. TEs also can be active during normal neurogenesis. By mining a series of deep sequencing datasets of protein-RNA interactions and of gene expression profiles, we uncovered extensive binding of TE transcripts to TDP-43, an RNA-binding protein central to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Second, we find that association between TDP-43 and many of its TE targets is reduced in FTLD patients. Third, we discovered that a large fraction of the TEs to which TDP-43 binds become de-repressed in mouse TDP-43 disease models. We propose the hypothesis that TE mis-regulation contributes to TDP-43 related neurodegenerative diseases.. ...
Researchers will present findings at the AANS Annual Scientific meeting of their studying testing if Intralaminar thalamic deep brain stimulation (ILN-DBS) could have an effect on dementia and other neurodegenerative diseases that cause severe cognitive dysfunction.
Recent studies in neurodegenerative conditions have increasingly highlighted that the same neuropathology can trigger different clinical phenotypes or, vice-versa, that similar phenotypes can be triggered by different neuropathologies. This evidence has called for the adoption of a pathology spectrum-based approach to study neurodegenerative proteinopathies. These conditions share brain deposition of abnormal protein aggregates, leading to aberrant biochemical, metabolic, functional, and structural changes. Positron emission tomography (PET) is a well-recognized and unique tool for the in vivo assessment of brain neuropathology, and novel PET techniques are emerging for the study of specific protein species. Today, key applications of PET range from early research and clinical diagnostic tools to their use in clinical trials for both participants screening and outcome evaluation. This position article critically reviews the role of distinct PET molecular tracers for different neurodegenerative ...
Current diagnostic approaches to neurodegenerative diseases are often flawed as they are often invasive and cannot effectively diagnose early-onset dementia. Antibody-based therapeutics for neurodegenerative diseases are very promising but often lack specificity to certain biomarkers and require invasive methods of administration such as a lumbar puncture. In this study I report a novel quantum-dot (QD) conjugated bispecific-antibody (BsAb) diagnosis system designed for Alzheimers disease. This structure is easy to synthesize and displays specificity to oligomeric amyloid-beta (Aβ), which is often present before Alzheimers symptoms starts to manifest. The bispecific antibody also binds with a weak affinity to transferrin receptors - thus potentially allowing it to cross the blood-brain barrier (BBB) via receptor-mediated transcytosis and reducing the necessity for extremely- invasive means of administration such as a lumbar puncture. The CdTe/ZnS QDs conjugated to the BsAb have multimodal, ...
The EU Joint Programme - Neurodegenerative Disease Research (JPND) has announced a EUR 30 million call for neurodegenerative disease research topped-up with EUR 10 million from the Horizon 2020 framework programme for research and innovation of the European Union.. Neurodegenerative diseases such as Alzheimers and Parkinsons are a truly global challenge. Most of these diseases remain incurable and are strongly linked with aging populations. Dementias alone affect more than 7 million people in Europe and their care is estimated to cost EUR 130 billion a year. The challenge facing the world of diagnosing, treating and caring for people affected by neurodegenerative diseases is extremely daunting and no single country alone has the expertise or resources necessary to tackle all of the big questions in this area.. JPND was established in 2009 to enable participating EU Member States to work together on the challenge of age-related neurodegenerative diseases, in particular Alzheimers. In the past ...
The role of lipids in autophagy and its implication in neurodegeneration - Neurodegenerative diseases are, at present, major socio-economic burdens without effective treatments and their increasing prevalence means that these diseases will be a challenge for future generations. Neurodegenerative diseases may differ in etiology and pathology but are often caused by the accumulation of dysfunctional and aggregation-prone proteins. Autophagy, a conserved cellular mechanism, deals with cellular stress and waste product build-up and has been shown to reduce the accumulation of dysfunctional proteins in animal models of neurodegenerative diseases. Historically, progress in understanding the precise function of lipids has traditionally been far behind other biological molecules (like proteins) but emerging works demonstrate the importance of lipids in the autophagy pathway and how the disturbance of lipid metabolism is connected to neurodegeneration. Here we review how altered autophagy and the disturbance of
The role of inflammation in the progression of neurodegenerative disease remains unclear. We have shown that systemic bacterial insults accelerate disease progression in animals and in patients with Alzheimers disease. Disease exacerbation is associated with exaggerated CNS inflammatory responses to systemic inflammation mediated by microglia that become primed by the underlying neurodegeneration. The impact of systemic viral insults on existing neurodegenerative disease has not been investigated. Polyinosinic:polycytidylic acid (poly I:C) is a toll-like receptor-3 (TLR3) agonist and induces type I interferons, thus mimicking inflammatory responses to systemic viral infection. In the current study we hypothesized that systemic challenge with poly I:C, during chronic neurodegenerative disease, would amplify CNS inflammation and exacerbate disease. Using the ME7 model of prion disease and systemic challenge with poly I:C (12 mg/kg i.p.) we have shown an amplified expression of IFN-alpha and ...
A type of white blood cell that is important to the immune system may provide hope for new therapies for amyotrophic lateral sclerosis (ALS), as well as other neurodegenerative diseases, according to a study published online today in the Proceedings of the National Academy of Sciences.
According to a new study, tetrahydrocannabinolic acid (THCA), a compound found in cannabis, may be useful in treating neurodegenerative and neuroinflammatory diseases.
GENETICS AND ENVIRONMENT. Each of the major neurodegenerative disorders may be familial in nature. HD is exclusively familial; it is transmitted by autosomal dominant inheritance, and the molecular mechanism of the genetic defect has been defined. Nevertheless, environmental factors importantly influence the age of onset and rate of progression of HD symptoms. PD, AD, and ALS are mostly sporadic without clear pattern of inheritance. But for each there are well-recognized genetic forms. For example, there are both dominant (α-synuclein, LRRK2) and recessive (parkin, DJ-1, PINK1) gene mutations that may give rise to PD. In AD, mutations in the genes coding for the amyloid precursor protein (APP) and proteins known as the presenilins (involved in APP processing) lead to inherited forms of the disease. Mutations in the gene coding for copper-zinc superoxide dismutase (SOD1) account for about 2% of the cases of adult-onset ALS. There are also genetic risk factors that influence the probability of ...
A drug that boosts activity in the brains garbage disposal system can decrease levels of toxic proteins associated with Alzheimers disease and other neurodegenerative disorders and improve cognition in mice, a new study by neuroscientists at Columbia University Medical Center has found.
Herbicides have been recognized as the main environmental factor associated with human neurodegenerative disorders such as Parkinsons disease(PD). Previous studies indicated that the exposure to glyphosate, a widely used herbicide, is possibly linked to Parkinsonism, however the underlying mechanis …
If you have a question about this talk, please contact Gabriella Heller.. Toxicity of misfolded proteins and mitochondrial dysfunction are key factors that promote age-associated functional neuronal decline and neurodegenerative disease across species. Although these neurotoxic challenges have long been considered to be cell-intrinsic, evidence now supports that misfolded human disease proteins originating in one neuron can be transferred to neighboring cells, a phenomenon proposed to promote pathology spread. Likewise, mitochondria can be sent out of the cell that made them for transcellular degradation by neighbors. We discovered and are characterizing a dramatic, but previously unknown, capacity of C. elegans adult neurons to extrude large (~5µM) vesicles that can include aggregated proteins (including human neurodegenerative disease proteins) and damaged mitochondria. Strikingly, extruded exopher contents can be found in both neighboring and remote cells. We suggest that throwing out the ...
Abstract: OBJECTIVE: While the causes of neuronal death in Parkinsons disease (PD) and other neurodegenerative disorders are still unknown, several mechanisms are under discussion: programmed vs. passive cell death (apoptosis vs. necrosis), mainly based on conflicting results on the rare presence or absence of DNA fragmentation in substantia nigra neurons using the in situ DNA-labeling (TUNEL) method. DESIGN/METHODS: In 4 cases of Parkinsons disease (PD), 2 cases of Dementia with Lewy bodies (DLB) and 3 age-matched controls, the TUNEL/ISEL method was used to detect DNA fragmentation in substantia nigra locus coeruleus and cerebral cortex [method by Gold et al. (1994)]. In addition, immunohistochemistry was performed for an array of apoptosis-related proteins, i.e. the recently described apoptosis specific protein cJun/AP1 (ASP), the proto-oncogenes c-Jun, c-Jun AP1, Bcl2, Bax, Bcl-x, p53, CD 95 (Fas/Apo-1), activated caspase 3, several heat shock proteins (alpha-B crystallin, ubiquitin), and ...
Significant progress has been made over the past two decades on the pathogenesis of individual neurodegenerative diseases, including Alzheimers disease (AD), amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). However, different neurodegenerative syndromes have been mainly studied mechanistically in isolation. There has been a lack of concerted effort to ascertain whether and how these pathogenic processes may be linked to one another. In the most recent issue of journal Acta Neuropathologica, Dr. Mingkuan Sun, William Bell and Katherine LaClair, co-first authored a report about cryptic exon incorporation in Alzheimers disease cases exhibiting TDP-43 pathology. This is the first evidence on how loss of TDP-43 function from neurons, a common shared feature with ALS and FTD, could contribute to pathogenesis of AD.. It has been known for years that other factors besides Aβ and tau also contribute to neurodegeneration and cognitive failure in AD. The most convincing evidence is ...
Bile acids, a structurally related group of molecules derived from cholesterol, have a long history as therapeutic agents in medicine, from treatment for primarily ocular diseases in ancient Chinese medicine to modern day use as approved drugs for certain liver diseases. Despite evidence supporting a neuroprotective role in a diverse spectrum of age-related neurodegenerative disorders, including several small pilot clinical trials, little is known about their molecular mechanisms or their physiological roles in the nervous system. We review the data reported for their use as treatments for neurodegenerative diseases and their underlying molecular basis. While data from cellular and animal models and clinical trials support potential efficacy to treat a variety of neurodegenerative disorders, the relevant bile acids, their origin, and the precise molecular mechanism(s) by which they confer neuroprotection are not known delaying translation to the clinical setting.
Jika anda yang sedang mencari informasi [beasiswa] [info] 8 PhD scholarships sponsored by the German Research Center for Neurodegenerative Diseases (DZNE), maka Beasiswa akan menyampaikan tentang [beasiswa] [info] 8 PhD scholarships sponsored by the German Research Center for Neurodegenerative Diseases (DZNE) seperti dibawah ini ...
Many neurodegenerative diseases are characterized by the early loss of select groups of cells in the brain, followed only later by more widespread degeneration. Understanding the cause of the enhanced vulnerability displayed by select cell groups may point towards the root causes of these diseases and lead to novel therapeutic targets. Professor Myriam Heimans lab studies the selective vulnerability and pathophysiology seen in two neurodegenerative diseases of the basal ganglia, Huntingtons disease and Parkinsons disease.. The easily recognizable ravages of Huntingtons disease and Parkinsons disease on normal motor control reflect the loss of either dopamine-producing cells (Parkinsons disease) or dopamine-receiving cells (Huntingtons disease) in the brain. Until fairly recently, patients afflicted with these diseases would be diagnosed mainly by these abnormal motor behaviors. However, it was not known why a patient was afflicted; no usually suspected causes existed. The last twenty ...
Central nervous system (CNS)-related disorders, including brain cancer, lysosomal storage disorders, traumatic brain or spinal cord injury, chronic pain, or chronic neurodegenerative diseases, such as Alzheimers, Parkinsons disease or Amyotrophic Lateral Sclerosis, still represent a major burden for the society and demand innovative and more efficacious therapeutic approaches. Indeed, delivering therapeutics to the CNS is challenging since the blood-brain barrier (BBB) must be overcome to gain access to brain cells. Moreover, monitoring drug biodistribution or target engagement is rarely feasible if not impossible, due to the limited accessibility to tissue sampling, with the exception of the cerebrospinal fluid (CSF). However, CSF analysis is not always fully informative, especially for those conditions where the analyte is not a soluble compound released in biological fluids.Recent advances in nanomaterial science and nanoparticles (NPs) technology have provided a great breakthrough in pharmacology
Nagoya, Japan - Frontotemporal lobar degeneration (FTLD) is a type of dementia that appears earlier in life than Alzheimers disease (AD). Both FTLD and AD, along with several other neurodegenerative diseases, are marked by the appearance and clustering of the protein tau in nerve cells. However, there is much left to be explored about this…
We primarily use human neurons made from induced pluripotent stem cells as well as in vitro protein aggregation models to delineate the pathogenic mechanisms of age-related neurodegenerative diseases such as Alzheimers and Parkinsons disease. Our group is largely focused on utilizing neurons from rare lysosomal diseases to study how alterations in biomolecule degradation pathways influence the accumulation and conformation of disease-linked proteins such as alpha-synuclein, abeta, and tau. Mechanistic insights gained from studies of rare lysosomal diseases are used as a way to view and elucidate the pathological mechanisms of common neurodegenerative diseases. Another major effort of the lab is to determine how amyloid formation influences cellular self-renewal mechanisms in neurons, such as lysosomal clearance of damaged macromolecules, and the effect on the aging process ...
Alzheimers disease (AD), also known as just Alzheimers, is a chronic neurodegenerative disease that usually starts slowly and gets worse over time. It is the cause of 60% to 70% of cases of dementia. The most common early symptom is difficulty in remembering recent events (short-term memory loss).[1] As the disease advances, symptoms can include problems with language, disorientation (including easily getting lost), mood swings, loss of motivation, not managing self care, and behavioural issues. As a persons condition declines, they often withdraw from family and society. Gradually, bodily functions are lost, ultimately leading to death. Although the speed of progression can vary, the average life expectancy following diagnosis is three to nine years.. The cause of Alzheimers disease is poorly understood. About 70% of the risk is believed to be genetic with many genes usually involved. Other risk factors include a history of head injuries, depression, or hypertension. The disease process is ...
Parkinsons disease is a chronic neurodegenerative disease that is characterized by the loss of dopamine-producing neurons in the substantia nigra region of the brain. There is also a simultaneous loss of norepinephrine-producing neurons in a region called the locus coeruleus. Administration of methyl phenyl tetrahydropyridine (MPTP) to laboratory animals is a common model for Parkinsons disease; however, MPTP does not cause the motor deficits seen in humans with Parkinsons disease. NIEHS-supported investigators tested mice to determine whether the loss of norepinephrine neurons was necessary for the motor deficits seen in Parkinsons disease. They used transgenic mice that totally lack norepinephrine altogether. The researchers detected no motor deficits in control mice treated with MPTP - despite an 80 percent reduction in the number of dopamine-producing cells. On the other hand, the norepinephrine-lacking mice exhibited motor deficits in most tests, along with other movement disorders, ...
Clinical trials for Alzheimers and other neurodegenerative diseases are expensive and slow, in part because they cannot find enough participants. Of the 5 million people with Alzheimers in the United States alone, many never learn about trial opportunities, and for rare dementias the pool of potential volunteers is small to begin with. Online registries that allow people to be contacted about trial opportunities in their area have sprung up to speed recruitment. Some registries educate members about the disease; others establish active patient communities (May 2011 news; Nov 2013 news). Most sites ask for minimal information at sign-up, usually name, email address, birth year, and zip code. After registering, people may choose to complete more detailed health questionnaires in order to be matched to trials. Most registries include people with or without a diagnosis. In Alzheimers, researchers are beginning to test treatments in people at pre-dementia stages. These participants are hard to ...
TY - JOUR. T1 - Neurodegenerative diseases and exposure to the environmental metals Mn, Pb, and Hg. AU - Charlet, Laurent. AU - Chapron, Yves. AU - Faller, Peter. AU - Kirsch, Regina. AU - Stone, Alan T.. AU - Baveye, Philippe C.. PY - 2012/10. Y1 - 2012/10. N2 - Metal ions appear to play an important role in several neurodegenerative (ND) diseases. Evidence suggests that metal ions bind directly to causative amyloidogenic proteins and modulate their aggregation into amyloids, considered to be a key event in the etiology of ND diseases. Apart from this well-documented binding of essential metals to amyloidogenic proteins, other, non-essential metal ions have been considered to be environmental hazards for neuronal disorders, but tight causative relations have yet to be established. The present article provides a review of the potential role of manganese, lead, and mercury as environmental risk factors in ND diseases, and covers in detail environmental availability of these metals, their uptake ...
Finding biomarkers that reflect the amount of peripheral nerve damage (peripheral neuropathies) and that the biomarker will quickly drop in value in response to to effective treatment are desired goals. The tools we need for developing biomarkers for equine neurodegenerative diseases are not available. These tools include a laboratory model for each neurodegenerative disease, putative treatments, and a money bin.. There is an alternate path leading to biomarker development and that is the horizon we are chasing. The biomarker quest project identifies natural cases of disease with neurodegeneration followed by evaluating the data from those cases. Sifting through the data is a process of eliminating the negative, selecting the positive, and interpreting the in-between. I hear a jingle in there somewhere! Generally diseases follow a typical course, or pathogenesis. Interpreting enough cases points toward the direction we should take and where to concentrate our assets. Often clues to a direction ...
The results described here support the conclusion that AT-1 is the ER membrane acetyl-CoA transporter and that its function is essential for the normal physiology of the cell. The recent identification of a missense mutation in AT-1 associated with SPG42 and the fact that AT-1 is upregulated in chronic neurodegenerative diseases such as sporadic ALS and late-onset (sporadic) AD point to a crucial role in disorders that are characterized by neuronal dysfunction and/or loss.. Although initially associated with nuclear and cytosolic proteins, covalent acetylation of the ε-amino group of lysine residues also occurs in the mitochondria (Schwer et al., 2006) and in the early secretory pathway (Costantini et al., 2007). In the secretory pathway, it appears to function as a new form of post-translational regulation of membrane proteins. We initially identified the acetylation machinery while analyzing the metabolism of BACE1 (Costantini et al., 2007). Following that initial finding, we have also shown ...
An increasing number of neurodegenerative diseases are being found to be associated with the abnormal accumulation of aggregated proteins in the brain. In Parkinsons disease, this process involves the aggregation of alpha-synuclein (α-syn) into intraneuronal inclusions. Thus, compounds that inhibit α-syn aggregation represent a promising therapeutic strategy as disease-modifying agents for neurodegeneration. The formation of α-syn amyloid aggregates can be reproduced in vitro by incubation of the recombinant protein. However, the in vitro aggregation of α-syn is exceedingly slow and highly irreproducible, therefore precluding fast high throughput anti-aggregation drug screening. Here, we present a simple and easy-to-implement in-plate method for screening large chemical libraries in the search for α-syn aggregation modulators. It allows us to monitor aggregation kinetics with high reproducibility, while being faster and requiring lower protein amounts than conventional aggregation assays. We
Pro-apoptotic signaling caused by down-modulation of KIAA0358 or expression of IG20-SV4 effectively induces spontaneous apoptosis and sensitization to TNFa-induced apoptosis in neuroblastoma cells. Methods and composition to enhance cell death in neuroblastoma are provided. Methods and compositions to reduce cell death in neurodegenerative disorders are provided.
Caution: Medical devices made by Scion NeuroStim have not received marketing authorization from the US Food & Drug Administration (FDA) for use in chronic neurodegenerative diseases such as Parkinsons Disease and are not available for sale. Neuromodulation medical devices made by Scion NeuroStim are covered by the following US patents, with other US and international patents pending ...
Environmental Factors in Neurodevelopmental and Neurodegenerative Disorders presents a state-of-the-art review of the effects of environmental contaminants on the development and degeneration of the human nervous system, brought together by world-leading experts in the field. Part One describes the adverse effects that the environment can have on neurological development, and how these effects may exhibit. Specific contaminants and their possible consequences of exposure are addressed (lead, methylmercury, alcohol), as well as specific disorders and the environmental factors associated with them, such as the effect of diet on attention deficit and hyperactivity disorders. Part Two tackles neurodegenerative disorders, specifically addressing their potential neurotoxic origins, and discussing the increasing interest in the effects that early exposure may have in later life. Environmental Factors in Neurodevelopmental and Neurodegenerative Disorders is an invaluable reference for those professionals
Destruction and death of neurons can lead to neurodegenerative diseases. One possible way to treat neurodegenerative diseases and damage of the nervous system is replacing damaged and dead neurons by cell transplantation. If new neurons can replace the lost neurons, patients may be able to regain the lost functions of memory, motor, and so on. Therefore, acquiring neurons conveniently and efficiently is vital to treat neurological diseases. In recent years, studies on reprogramming human fibroblasts into neurons have emerged one after another, and this paper summarizes all these studies. Scientists find small molecules and transcription factors playing a crucial role in reprogramming and inducing neuron production. At the same time, both the physiological microenvironment in vivo and the physical and chemical factors in vitro play an essential role in the induction of neurons. Therefore, this paper summarized and analyzed these relevant factors. In addition, due to the unique advantages of physical
The neurotransmitter glutamate has been implicated in multiple neurodegenerative studies. Researchers agree that glutamate excitotoxicity undoubtedly has a role in the pathogenesis of Alzheimer disease, the most common neurodegenerative pathology affecting the elderly population. Research suggests glutamate excitotoxicity accelerates the progression of Alzheimer disease.[12] Glutamate is also implicated in the pathogenesis of Parkinson disease. Mutations in genes encoding the parkin and DJ1 proteins are present in Parkinsons disease, which are involved in the regulation of excitatory glutamate synapses. These proteins may also protect neurons against glutamate excitotoxicity.[13][14]. Gamma-aminobutyric acid (GABA), the major inhibitory neurotransmitter in the central nervous system, is targeted in the treatment of anxiety disorder, insomnia, epilepsy, and other conditions. In particular, these drugs alter GABAergic function by targeting the GABAA and GABAB receptors.[15]. Not only does ...
Neurodegenerative diseases such as Alzheimer�s disease (AD) and Parkinson�s disease (PD) are currently considered as protein misfolding diseases. Determina...
People who suffer from REM sleep behavior disorder are at increased risk of developing Parkinsons disease and dementia as they age, a new study reports. Researchers report RBD causes a lack of dopamine in the brain, and this can contribute to the development of neurodegenerative diseases.... Read More... ...
By Eric Sauter Scientists from the Florida campus of The Scripps Research Institute have uncovered a potentially important new therapeutic target that could prevent stress-related cell death, a characteristic of neurodegenerative diseases such as Parkinsons, as well as heart attack and stroke. In the study, published recently in the journal ACS Chemical Biology, the scientists showed they could disrupt a specific interaction of a critical enzyme that would prevent cell death without harming other important enzyme functions. The enzyme in question is c-jun-N-terminal kinase (JNK), pronounced junk, which has been implicated in many processes in the bodys response to stresses, such as oxidative stress, protein misfolding, and metabolic disorder. JNK also plays an important role in nerve cell survival and has become a target for drugs to treat neurodegenerative disorders such as Parkinsons disease. In recent studies, JNK has been found to migrate to the mitochondria-the part of the cell that ...
Notification may be sought. Participants may additionally consent to banking their DNA for use in future genetic research studies into neurodegenerative diseases. The blood drawn for use in the main study will also be used for extracting the DNA that will be stored for use in future research. Alternatively, specific studies on these samples may be abandoned, but data will be kept for 5 years after publication. The biospecimens and data have been provided for genetic research, for clinical, pathologic and genetic correlations, to try and identify the molecular basis of Parkinson Syndrome and related neurodegenerative disorders. The biospecimens may be destroyed or returned to collaborating Institutions, at their request and at any time. Similarly, individuals have the rights to withdraw their participation. The specific collaborating Institution only needs to notify Dr. Farrer (or the database administrator) to remove a record/sample.. ...
... or neurodegenerative diseases such as Parkinson's disease and Alzheimer's disease. These conditions can cause anosmia. In ... Other neurodegenerative diseases that affect olfactory dysfunction include Huntington's disease, multi-infarct dementia, ... dysfunction is a cardinal feature of several neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease. ... Neurodegenerative diseases[edit]. Neurologists have observed that olfactory ...
Neurodegenerative diseases[edit]. Many neurodegenerative diseases can cause memory loss. Some of the most prevalent (and, as a ... including memory loss and may help protect long-term and visiospatial memory from neurodegenerative disease.[32] ... more serious problems with memory occur due to traumatic brain injury or neurodegenerative disease. ... Huntington's disease, multiple sclerosis, Parkinson's disease, and schizophrenia. None act specifically on memory; instead, ...
Many neurodegenerative diseases including Parkinson's, Alzheimer's, and Huntington's occur as a result of neurodegenerative ... Neurodegenerative diseases[edit]. Neurodegeneration is the umbrella term for the progressive loss of structure or function of ... and problems acquired later in life through acquired brain injuries or neurodegenerative diseases like dementia. These ... resulting in long-term decline due to damage or disease in the body. In the early stages of Alzheimer's disease, whose symptoms ...
Examples of neurodegenerative disorders include Alzheimer's disease, Parkinson's disease, and Huntington's disease. The focus ... including neurodegenerative disorders such as Parkinson's disease (PD) and Alzheimer's disease (AD), in rodent and non-human ... "Neurodegenerative Diseases". National Institute of Environmental Health Sciences. Retrieved 2019-02-27.. ... Neurodegenerative disorders are a result of neuronal loss of function over time which lead to cell death. ...
Neurodegenerative Disease. 5 (3-4): 261-263. doi:10.1159/000113719. PMID 18322407. v t e. ... Moon, G.; Manktelow, T.C. (2002). "Cognitive deficits in recently diagnosed untreated patients with Parkinson's disease". ... disease (both physical and mental), medicines and drugs. The standard battery of cognitive tests in The CDR system includes ... Alzheimer's disease, and Vascular Dementia". Neurology. 54 (8): 1616-1625. doi:10.1212/WNL.54.8.1616. PMID 10762503. Zhang, K; ...
"Alumnus Jeffrey Kordower, Founding Director of the ASU-Banner Neurodegenerative Disease Research Center, on How He Got His ... "Neurodegenerative Disease". Biodesign Institute , ASU. Retrieved 2021-04-07. "Team". Biodesign Institute , ASU. Retrieved 2021- ... The center is pursuing new battleground tactics in the war against Alzheimer's and other neurodegenerative diseases. The center ... Dysfunctional mitochondria are associated with Parkinson's disease, Alzheimer's disease, amyotrophic lateral sclerosis, ataxias ...
Best known for her work on the signaling of acetylcholine and its relevance in stress responses and neurodegenerative diseases ... neuromuscular and neurodegenerative (Parkinson's and Alzheimer's) diseases. She is the author of over 280 peer-reviewed journal ... Neurodegenerative Diseases. 9 (2): 87-98. doi:10.1159/000331328. PMID 22042332. S2CID 24015350. Gabison, Yoram (14 November ... "Cholinergic-associated loss of hnRNP-A/B in Alzheimer's disease impairs cortical splicing and cognitive function in mice". EMBO ...
397-. ISBN 978-3-642-02912-7. Dominguez C (18 November 2010). Neurodegenerative Diseases. Springer Science & Business Media. pp ... gastroesophageal reflux disease, and dental anxiety. It reached phase II clinical trials for all of the aforementioned ...
Neurodegenerative Diseases. 8 (5): 375-380. doi:10.1159/000324373. ISSN 1660-2862. PMC 3121545. PMID 21389683. Huang, S.; ... chronic kidney disease,HIV-1 disease,tick-borne encephalitis and Lyme,malaria,hepatitis, burns,multiple organ dysfunction ... Decreased levels are often associated with ill health and disease. A growing list of insults showing loss of pGSN includes ... Ji, Lina; Zhao, Xi; Hua, Zichun (2015-01-06). "Potential Therapeutic Implications of Gelsolin in Alzheimer's Disease". Journal ...
Neuro-Degenerative Diseases. 3 (4-5): 275-83. doi:10.1159/000095267. PMID 17047368. S2CID 17324271. Francis R, McGrath G, Zhang ...
Neuro-Degenerative Diseases. 4 (5): 366-75. doi:10.1159/000105157. PMID 17622779. S2CID 9020464. Lescuyer P, Allard L, ... who have found emerging evidence that indicates a role in differentiating between different neurodegenerative diseases. To ... is decreased in brains of patients with Down syndrome and Alzheimer's disease. Journal of Neural Transmission. Supplementum. ...
Shen J (2 October 2013). "Function and dysfunction of presenilin". Neuro-Degenerative Diseases. 13 (2-3): 61-3. doi:10.1159/ ... Smialowska A, Baumeister R (2006). "Presenilin function in Caenorhabditis elegans". Neuro-Degenerative Diseases. 3 (4-5): 227- ... An important part of the disease process in Alzheimer's disease is the accumulation of Amyloid beta (Aβ) protein. To form Aβ, ... onset forms of familial Alzheimer's disease by Peter St George-Hyslop at the Centre for Research in Neurodegenerative Diseases ...
Neurodegenerative Disease Management. 2 (6): 561-564. doi:10.2217/nmt.12.64. Laws, Keith R; Stoet, Gijsbert; O'Connor, Daryl B ... Laws, Keith R; Irvine, Karen (December 2012). "Do women with Alzheimer's disease demonstrate greater cognitive deterioration ... "Greater cognitive deterioration in women than men with Alzheimer's disease: a meta analysis". Human Psychopharmacology: ... in patients with schizophrenia and to demonstrate worse cognitive outcomes in women suffering from Alzheimer's disease. Laws' ...
2002). "Is the saitohin gene involved in neurodegenerative diseases?". Ann. Neurol. 52 (6): 829-32. doi:10.1002/ana.10384. PMID ... Neuro-Degenerative Diseases. 2 (1): 28-35. doi:10.1159/000086428. PMID 16909000. S2CID 6644618. v t e. ... which is nested in the tau locus and confers allele-specific susceptibility to several neurodegenerative diseases, interacts ... 2006). "TAU haplotype and the Saitohin Q7R gene polymorphism do not influence CSF Tau in Alzheimer's disease and are not ...
... voice and language in Parkinson's disease: Changes and interventions". Neurodegenerative Disease Management. 2 (3): 279-289. ... Parkinson's disease is a chronic neurodegenerative disorder that involves the loss of dopaminergic neurons in the brain. While ... They have concluded that patients with Parkinson's disease tend to struggle with specific areas of prosody; they are less able ... The degradation of prosody in Parkinson's disease over time is independent of motor control issues, and is thus separate from ...
Neuro-Degenerative Diseases. 11 (3): 129-40. doi:10.1159/000336427. PMID 22626981. S2CID 46024586. Giménez-Llort L, Ratia M, ... In animal studies it has nootropic and neuroprotective effects, and is used in research into Alzheimer's disease, and although ... "Huprine X is a novel high-affinity inhibitor of acetylcholinesterase that is of interest for treatment of Alzheimer's disease ... "Huprine X and huperzine A improve cognition and regulate some neurochemical processes related with Alzheimer's disease in ...
February 2016). "Emerging therapies in Friedreich's ataxia". Neurodegenerative Disease Management. 6 (1): 49-65. doi:10.2217/ ... The disease primarily affects the spinal cord and peripheral nerves. The spinal cord becomes thinner and nerve cells lose some ... The disease evolves differently in different people. In general, those diagnosed at a younger age or with longer GAA triplet ... The disease is progressive, with increasing staggering or stumbling gait and frequent falling. By the third decade, affected ...
... as well as diseases such as neurodegenerative diseases, atherosclerosis, and cancer. To elaborate, LRP1 mainly contributes to ... and diseases, such as neurodegenerative diseases, atherosclerosis, and cancer. The LRP1 gene encodes a 600 kDa precursor ... Neuro-Degenerative Diseases. 11 (1): 13-21. doi:10.1159/000337231. ISSN 1660-2862. PMID 22572854. S2CID 30189180. Bachmeier, ... In support of this, LRP1 expression is reduced in endothelial cells as a result of normal aging and Alzheimer's disease in ...
Neurodegenerative Disease Management. 7 (5): 331-342. doi:10.2217/nmt-2017-0017. PMID 29043889. Burman, Joachim; Tolf, Andreas ... The Lewis-Sumner form of this condition is considered a rare disease with only 50 cases reported up to 2004. A total of 90 ... In some case EMG/NCV can be normal). Serum test to exclude other autoimmune diseases. Lumbar puncture and serum test for anti- ... These antibodies are present in the branch of CIDP diseases comprised by anti-GM1, anti-GD1a, and anti-GQ1b. Sural nerve biopsy ...
"List of drugs in development for neurodegenerative diseases. Update June 2007". Neuro-Degenerative Diseases. 4 (6): 443-86. doi ... It was under development by Dainippon Sumitomo Pharma for the treatment of Alzheimer's disease and made it to phase II clinical ... "Current Strategies of Therapy in Alzheimer's Disease" (PDF). The Open Neuropsychopharmacology Journal. 1: 19-23. doi:10.2174/ ...
Neuro-Degenerative Diseases. 17 (6): 242-250. doi:10.1159/000478741. PMID 28787714. S2CID 1772982. Lashuel HA, Hartley DM, ... Citron M (September 2004). "Strategies for disease modification in Alzheimer's disease". Nature Reviews. Neuroscience. 5 (9): ... "Familial Alzheimer's disease with the amyloid precursor protein position 717 mutation and sporadic Alzheimer's disease have the ... However, familial Alzheimer's disease is likely to result from altered proteolytic processing. This is evidenced by the fact ...
"List of drugs in development for neurodegenerative diseases". Neuro-Degenerative Diseases. 1 (1): 50-70. doi:10.1159/000077879 ... It was studied by Aventis for the treatment of Alzheimer's disease, but was never marketed. Piracetam "The Use of Stems in the ...
Parish, CL; Arenas, E (2007). "Stem-cell-based strategies for the treatment of Parkinson's disease". Neuro-Degenerative ... for example for treating a number of neurological disorders such as Parkinson's disease and Huntington's disease. MSCs are ... However, in many diseases and disorders, cell are compromised by e.g. senescence, limited blood supply (ischemia), inflammation ... As such, in recent times, cell therapy has been recognized as an important field in the treatment of human disease, and ...
Neuro-Degenerative Diseases. 3 (4-5): 275-83. doi:10.1159/000095267. PMID 17047368. S2CID 17324271. Zhang YW, Luo WJ, Wang H, ... The protein was named after the Italian country Nicastro, reflecting the fact that Alzheimer's disease was described in 1963 ... Feldman RG, Chandler KA, Levy LL, Glaser GH (Oct 1963). "Familial Alzheimer's disease". Neurology. 13 (10): 811-24. doi:10.1212 ... Neurobiology of Disease. 14 (2): 194-204. CiteSeerX 10.1.1.624.6715. doi:10.1016/S0969-9961(03)00123-2. PMID 14572442. S2CID ...
Laboratories: Anderson lab, post-transcriptional control & inflammatory response Zhou lab, neurodegenerative disease Morimoto ... Neuro-Degenerative Diseases. 17 (6): 292-303. doi:10.1159/000480085. PMID 29035885. S2CID 40561105. Marrone L, Poser I, Casci I ... "TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types". Scientific Reports. 8 (1): 7551 ... Fathinajafabadi A, Pérez-Jiménez E, Riera M, Knecht E, Gonzàlez-Duarte R (2014). "CERKL, a retinal disease gene, encodes an ...
Neuro-Degenerative Diseases. 2 (6): 277-83. doi:10.1159/000092315. PMID 16909010. S2CID 45002038. Neve RL, McPhie DL (Apr 2007 ... Koo EH (Nov 2002). "The beta-amyloid precursor protein (APP) and Alzheimer's disease: does the tail wag the dog?". Traffic. 3 ( ... Maynard CJ, Bush AI, Masters CL, Cappai R, Li QX (Jun 2005). "Metals and amyloid-beta in Alzheimer's disease". International ... Tickler AK, Wade JD, Separovic F (Aug 2005). "The role of Abeta peptides in Alzheimer's disease". Protein and Peptide Letters. ...
Neurodegenerative Disease Management. 2 (1): 107-115. doi:10.2217/nmt.11.66. Grill, Joshua D.; Galvin, James E. (2014). " ... "Facilitating Alzheimer Disease Research Recruitment:". Alzheimer Disease & Associated Disorders. 28 (1): 1-8. doi:10.1097/WAD. ... The service is one of many efforts in the field of Alzheimer's disease research to recruit research participants. Alzheimer's ... Webstudy for a Trial-Ready Cohort for Preclinical and Prodromal Alzheimer's Disease (TRC-PAD)". doi:10.14283/jpad.2020.46. Cite ...
Neuro-Degenerative Diseases. 3 (4-5): 275-83. doi:10.1159/000095267. PMID 17047368. S2CID 17324271. Luo WJ, Wang H, Li H, Kim ... whose misfolded form is implicated in the causation of Alzheimer's disease. All of the components of the gamma-secretase ...
Parish, CL; Arenas, E (2007). "Stem-cell-based strategies for the treatment of Parkinson's disease". Neuro-Degenerative ... neurodegenerative disorders (such as Parkinson's disease), AIDS, etc. In addition to their potential in regenerative medicine, ... In addition, this will allow the generation of ES cell lines from patients with a variety of genetic diseases and will provide ... Potential uses include the treatment of diabetes and heart disease. The cells are being studied to be used as clinical ...
"List of drugs in development for neurodegenerative diseases. Update September 2005". Neuro-Degenerative Diseases. 2 (2): 61-108 ... Alzheimer's disease, Parkinson's disease, and nicotine dependence. Though initially studied as a potential treatment for a ...
Crohn's disease, HIV, or other autoimmune and neurodegenerative diseases. As all people with later-stage infection will have a ... "Lyme disease rashes and look-alikes". Lyme Disease. Centers for Disease Control and Prevention. 21 December 2018. Archived from ... "Lyme Disease Data and surveillance". Lyme Disease. Centers for Disease Control and Prevention. 5 February 2019. Archived from ... Treatment regimens for Lyme disease range from 14 days in early localized disease, to 14-21 days in early disseminated disease ...
... do cyanobacteria contribute to neurodegenerative disease?". Environmental Health Perspectives. 120 (3): a110-a116. doi:10.1289/ ... Parkinson's Disease and Alzheimer's Disease.[3] There is also an interest in the military potential of biological neurotoxins ... Exposure to the cyanobacteria neurotoxin BMAA may be an environmental cause of neurodegenerative diseases such as ALS, ... Are there implications for human neurodegenerative disease?" Informa Healthcare, 10(s2): 74-78. ...
Some diseases associated with mutations in the TATA box include gastric cancer, spinocerebellar ataxia, Huntington's disease, ... Several neurodegenerative disorders are associated TATA box mutations.[40] Two disorders have been highlighted, spinocerebellar ... Diseases[edit]. Mutations in the TATA box region affects the binding of the TATA-binding protein (TBP) for transcription ... "The American Review of Respiratory Disease. 118 (3): 635-6. doi:10.1128/mcb.10.8.3859. PMC 360896. PMID 2196437.. ...
In 2014, Phase 3 trials begin for drug to treat spinal muscular atrophy (SMA), a neurodegenerative disease, based on Adrian ... small subset of protein-coding genes within the much larger genome-now a mainstay of identifying genetic mutations in disease;[ ... studies cognition in the normal brain as a baseline for understanding dysfunction in psychiatric and neurodegenerative ...
"Neurodegenerative Diseases and Fasting". Antiaging-europe.com. Archived from the original on 2010-10-16. Retrieved 2010-10-18. ... Modern Nutrition in Health and Disease. Lippincott Williams & Wilkins. ISBN 9780781741330. Retrieved 22 January 2017.. ... intermittent fasting with a sensible water intake can strengthen the organism and assist thwarting degenerative diseases.[58] ...
"Mortality From Neurodegenerative Diseases in a Cohort of US Flight Attendants". American Journal of Industrial Medicine. 59 (7 ... There is also much concern regarding the transmission of contagious diseases, particularly tuberculosis. An open question ... "Association between respiratory tract diseases and secondhand smoke exposure among never smoking flight attendants: a cross- ...
... underlying selective neuronal death in neurodegenerative diseases such as Alzheimer's disease. Further this research involves a ... Discoveries concerning pathogenic mechanisms in Alzheimer's disease and other diseases. Awards. Denham Harman Research Award, ... "JOURNAL OF ALZHEIMER'S DISEASE. Retrieved 2018-05-14.. *^ "Archived copy". Archived from the original on 2009-09-12. Retrieved ... Smith served as Editor-in Chief of Journal of Alzheimer's Disease and also sat on the Editorial Board of over 20 leading ...
... or Corino de Andrade's disease,[1] is an autosomal dominant[2] neurodegenerative disease. It is a form of amyloidosis, and was ... "Rare-Disease Treatment From Alnylam to Cost $450,000 a Year". www.bloomberg.com. Retrieved 11 August 2018.. ... This disease is endemic in Portuguese locations Póvoa de Varzim and Vila do Conde (Caxinas), with more than 1000 affected ... In northern Sweden, more specifically Skellefteå (it is locally called "Skelleftesjukan", the Skellefteå disease), 1.5% of the ...
"Stem-cell-based strategies for the treatment of Parkinson's disease". 》Neuro-Degenerative Diseases》 4 (4): 339-347. ISSN 1660- ... "Designer's microglia with novel delivery system in neurodegenerative diseases". 》Medical Hypotheses》 83 (4): 510-512. ISSN 0306 ...
"BDNF-based synaptic repair as a disease-modifying strategy for neurodegenerative diseases". Nature Reviews. Neuroscience. 14 (6 ... Zuccato C, Cattaneo E (June 2009). "Brain-derived neurotrophic factor in neurodegenerative diseases". Nature Reviews. Neurology ... Alzheimer's disease,[71] Huntington's disease,[72] Rett syndrome,[73] and dementia,[74] as well as anorexia nervosa[75] and ... of humans with various neurodegenerative disease had all failed.[78] Schizophrenia[edit]. See also: Epigenetics of ...
... is a DNA sequence that causes disease or is associated with susceptibility to disease. They can be used to create genetic maps ... but is also a progressive and delayed neurodegenerative process made up of multiple, parallel, interacting, and interdependent ... It can also be a substance whose detection indicates a particular disease state, for example, the presence of an antibody may ... a biomarker indicates a change in expression or state of a protein that correlates with the risk or progression of a disease, ...
... and other neurodegenerative diseases. Researchers suggest that these cells possess a unique ability to remyelinate injured ... Transplantation of stem cells is also known to cause toxicity and graft-versus-host disease (GVHD). Apoptotic cells have been ... Transplantation of OECs into the spinal cord has become a possible therapy for spinal cord damage and other neural diseases in ... trials are currently being conducted to obtain more information on spinal cord injuries and other neurodegenerative diseases. ...
ALS is the most common motor neuron disease in adults and the third most common neurodegenerative disease[22] after Alzheimer's ... Disease Primers. 3 (17071): 17071. doi:10.1038/nrdp.2017.71. PMID 28980624.. *^ a b c d e f g h i j k l m n o p q r s t u v van ... Amyotrophic lateral sclerosis (ALS), also known as motor neurone disease (MND) or Lou Gehrig's disease, is a specific disease ... Other names for ALS include Charcot's disease, Lou Gehrig's disease, and motor neurone disease.[1] Amyotrophic comes from the ...
... diseases, muscular dystrophies[129] and several rare forms of neurodegenerative diseases associated with dementia.[130] As part ... and Pick's disease,[127] amyotrophic lateral sclerosis (ALS),[127] Huntington's disease,[126] Creutzfeldt-Jakob disease,[128] ... It may lead to brain tumors such as astrocytomas.[95] In some of the late-onset neurodegenerative diseases that share ... Such protein accumulation may contribute to the pathogenesis and phenotypic characteristics in neurodegenerative diseases,[118] ...
Neurodegenerative disordersEdit. *Huntington disease. No reliable conclusions could be drawn regarding the effectiveness of THC ... Parkinson's disease. Based on a single study, oral CBD extract was rated probably ineffective in treating levodopa-induced ... Alzheimer's disease. A 2009 Cochrane Review found insufficient evidence to conclude whether cannabis products have any utility ... Epidiolex (prescription form of purified cannabidiol derived from hemp used for treating some rare neurological diseases) ...
... and metals and their roles in aging and neurodegenerative diseases". Ann. N. Y. Acad. Sci. 1056: 430-49. Bibcode:2005NYASA1056 ... Citron M (September 2004). "Strategies for disease modification in Alzheimer's disease". Nat. Rev. Neurosci. 5 (9): 677-85. ... "The Alzheimer's Disease-Associated Amyloid β-Protein Is an Antimicrobial Peptide". PLoS ONE 5 (3): e9505. Bibcode:2010PLoSO... ... Implications for the role of amyloid-beta 1-42 in Alzheimer's disease". J. Biol. Chem. 271 (50): 32185-91. PMID 8943274. doi: ...
"The neurodegenerative disease protein aprataxin resolves abortive DNA ligation intermediates". Nature 443 (7112): 713-6. PMID ...
Coenzyme Q10 effects in neurodegenerative disease«, Neuropsychiatric Disease and Treatment,6. November 2009 ...
Parkinson disease[edit]. Parkinson disease is a neurodegenerative disorder partially caused by the cell death of brain and ... Mitochondria is involved in Parkinson's disease. In idiopathic Parkinson's disease, the disease is commonly caused by ... "Autophagy in Stress, Development & Disease, 2003, Gordon Research Conference".. *^ "Autophagy in Health and Disease (Z3), 2007 ... There are several genetic mutations implicated in the disease, including loss of function PINK1 [89] and Parkin.[90] Loss of ...
Parkinson disease[edit]. Parkinson disease is a neurodegenerative disorder partially caused by the cell death of brain and ... Mitochondria is involved in Parkinson's disease. In idiopathic Parkinson's disease, the disease is commonly caused by ... "Autophagy in Stress, Development & Disease, 2003, Gordon Research Conference".. *^ "Autophagy in Health and Disease (Z3), 2007 ... "Cell Death & Disease. 1 (1): e10. doi:10.1038/cddis.2009.8. PMC 3032517. PMID 21364612.. ...
... increase of alpha-synuclein aggregates in neurodegenerative diseases with tau-based inclusions". Archives of Neurology. 61 (12 ... new lesions in diffuse Lewy body disease, Pick's disease, and corticobasal degeneration. Journal of Neural Transmission. ... In the novel The Almost Sisters by Joshilyn Jackson, one of the main characters has the disease. Academy Award winning actor ... Lewy bodies are abnormal aggregates of protein that develop inside nerve cells, contributing to Parkinson's disease (PD), the ...
Neurodegenerative diseases of the central nervous system[edit]. Alzheimer's Disease (AD)[edit]. Main article: Alzheimer's ... 3 Neurodegenerative diseases of the central nervous system *3.1 Alzheimer's Disease (AD) *3.1.1 Epigenetic factors ... Disease: amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), Huntington's disease (HD), spinal muscular atrophy (SMA ... Neurodegenerative diseases of motor neurons[edit]. Amyotrophic lateral sclerosis (ALS)[edit]. Main article: Amyotrophic Lateral ...
Bovine spongiform encephalopathy (mad-cow disease) - fatal neurodegenerative disease in cows, which can be transmitted to ... Prusiner SB (2001). "Shattuck lecture--neurodegenerative diseases and prions". N Engl J Med. 344 (20): 1516-26. doi:10.1056/ ... The misfolded version PrPSc is associated with a variety of cognitive disorders and neurodegenerative diseases such as in ... Ross CA, Poirier MA (July 2004). "Protein aggregation and neurodegenerative disease". Nat. Med. 10 Suppl (7): S10-7. doi: ...
Å and its implications for inherited neurodegenerative diseases". Nat. Struct. Biol. 4: 578-585. PMID 9228951.. CS1 одржавање: ...
PSP may be mistaken for other neurodegenerative diseases such as Parkinson's disease and Alzheimer's disease. The cause of the ... US: The Foundation for PSP, CBD and Related Brain Diseases[47]. References[edit]. *^ a b c d e f g Golbe LI (April 2014). " ... Others consider them separate diseases.[16][17][18] PSP has been shown occasionally to co-exist with Pick's disease.[19] ... or as Alzheimer's disease because of the behavioral changes. It is one of a number of diseases collectively referred to as ...
... of neurodegenerative diseases. *Lamarckism. *Nutriepigenomics. *Position-effect variegation. *Preformationism. * ... Robertson KD, Wolffe AP (October 2000). "DNA methylation in health and disease". Nature Reviews Genetics. 1 (1): 11-9. PMID ... Ballestar E (2010). "Epigenetics lessons from twins: prospects for autoimmune disease". Clin Rev Allergy Immunol. 39 (1): 30-41 ... While age is a known risk factor for many diseases, age-related methylation has been found to occur differentially at specific ...
... is a potent neurotoxin associated with neurodegenerative disease,[12] and research has suggested a connection between ... but there is no medical evidence it is effective for treating cancer or any disease.[7] ... consumption of soursop and atypical forms of Parkinson's disease due to high concentrations of annonacin.[15][16][17][18] The ...
Examples include Gaucher disease, Fabry disease, Mucopolysaccharidoses and Glycogen storage disease type II. Such treatments ... muscular dystrophy/neurodegenerative disorder clinics). ... Allelic architecture of disease[edit]. Main article: Population ... Pritchard JK (2002). "The allelic architecture of human disease genes: common disease-common variant...or not?". Hum Mol Genet ... Sometimes the link between a disease and an unusual gene variant is more subtle. The genetic architecture of common diseases is ...
1993). „Substance P and substance P receptor histochemistry in human neurodegenerative diseases.". Regul. Pept. 46 (1-2): 174- ...
... affect millions of people worldwide, and Alzheimers disease and Parkinsons disease are the most ... Neurodegenerative diseases occur when nerve cells in the brain or peripheral nervous system lose function over time and ... That is, a person might have a gene that makes them more susceptible to a certain neurodegenerative disease, but whether, when ... This creates a critical need to improve our understanding of what causes neurodegenerative diseases and develop new approaches ...
Neurodegenerative diseases of the central nervous system[edit]. Alzheimers Disease (AD)[edit]. Main article: Alzheimers ... 3 Neurodegenerative diseases of the central nervous system *3.1 Alzheimers Disease (AD) *3.1.1 Epigenetic factors ... Disease: amyotrophic lateral sclerosis (ALS), Alzheimers disease (AD), Huntingtons disease (HD), spinal muscular atrophy (SMA ... Neurodegenerative diseases of motor neurons[edit]. Amyotrophic lateral sclerosis (ALS)[edit]. Main article: Amyotrophic Lateral ...
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Pages in category "Deaths from neurodegenerative disease". The following 8 pages are in this category, out of 8 total. This ... Retrieved from "https://en.wikipedia.org/w/index.php?title=Category:Deaths_from_neurodegenerative_disease&oldid=827035522" ...
Here we review the current state of rodent models for Alzheimers disease, Parkinsons disease, frontotemporal dementia, and ... Parkinsons disease, frontotemporal dementia, and amyotrophic lateral sclerosis. Nevertheless, our understanding of these ... benefit from more rigorous use of the models and from generation of animals that more faithfully recapitulate human disease. ... Animal models of adult-onset neurodegenerative diseases have enhanced the understanding of the molecular pathogenesis of ...
Roles of sigma-1 receptors on mitochondrial functions relevant to neurodegenerative diseases The sigma-1 receptor (Sig-1R) is a ... Parkinsons disease (PD) is the most common movement disorder and manifests as resting tremor, rigidity, bradykinesia, and ... LRRK 2 gene mutations in the pathophysiology of the ROCO domain and therapeutic targets for Parkinsons disease: a review ... promising experimental models for brain development and neurodegenerative disorders Three-dimensional (3D) brain organoids ...
... which causes this newly discovered disease, as well as related neurodegenerative diseases like Alzheimers Disease -- that ... Huntingtons disease is an hereditary neurodegenerative disease featured by alterations in movement, cognitive deficiency and ... Biomarker for earlier diagnosis of neurodegenerative diseases detected in the eye A new study led by Boston Medical Center ... Researchers identify CLCN6 as disease gene for severe lysosomal neurodegenerative disorder A mutation in the CLCN6 gene is ...
Study reveals the implication of lysosomes in the spread of Parkinsons disease Over the last few decades, neurodegenerative ... Huntingtons Disease (HD) is a progressive neurodegenerative condition characterized by motor, cognitive, and psychiatric ... Non-toxic derivative of tetanus neurotoxin may help treat depression, neurodegenerative diseases Depression has been treated ... Researchers worldwide are making a strong effort to understand neurodegenerative diseases pathogenesis, which is essential to ...
... uses genetic data from patient blood samples can strongly predict the progression and severity of neurodegenerative diseases ... "We dont have good biomarkers for neurodegenerative disease," he said. "So a second aim of the study was to see if we can take ... "Thats very important because there has been a lot of emphasis on finding brain biomarkers of neurodegenerative diseases like ... Cite this: AI Blood Test Predicts Neurodegenerative Disease Progression, Severity - Medscape - Feb 07, 2020. ...
... which plays a central role in two distinct neurodegenerative diseases. ... Neurodegenerative diseases: Deadly droplets. Ludwig-Maximilians-Universität München. Journal. Cell. Keywords. *MEDICINE/HEALTH ... which plays a central role in two distinct neurodegenerative diseases.. Aggregation of the DNA/RNA-binding protein FUS (FUsed ... Amyotrophic lateral sclerosis (ALS) is a motor neuron disease, which leads to progressive muscle weakness and ultimately to ...
An accumulation of protein clumps is known to occur in a number of neurodegenerative diseases, including Alzheimers disease, ... "Brain enzyme could prevent Alzheimers, neurodegenerative disease." Medical News Today. MediLexicon, Intl., 3 Jun. 2016. Web. ... Overall, the researchers believe their finding could pave the way for new treatments for a number of neurodegenerative diseases ... Whiteman, H. (2016, June 3). "Brain enzyme could prevent Alzheimers, neurodegenerative disease." Medical News Today. Retrieved ...
... Guest Editors: Mark A. Smith, George Perry, Xiongwei Zhu, and Abdelali ... Neurodegenerative Diseases: Mechanisms and Therapies, Mark A. Smith, George Perry, Xiongwei Zhu, and Abdelali Haoudi Editorial ... Oxidative Damage to RNA in Neurodegenerative Diseases, Akihiko Nunomura, Kazuhiro Honda, Atsushi Takeda, Keisuke Hirai, ... Signaling, Polyubiquitination, Trafficking, and Inclusions: Sequestosome 1/p62s Role in Neurodegenerative Disease, Marie W. ...
From Prion Diseases to Prion-Like Propagation Mechanisms of Neurodegenerative Diseases, Isabelle Acquatella-Tran Van Ba, ... Protein Misfolding and Neurodegenerative Diseases. Guest Editors: Alessio Cardinale, Roberto Chiesa, and Michael Sierks * ... Protein Misfolding and Neurodegenerative Diseases, Alessio Cardinale, Roberto Chiesa, and Michael Sierks Editorial (2 pages), ... Disulfide Bonding in Neurodegenerative Misfolding Diseases, Maria Francesca Mossuto Review Article (7 pages), Article ID 318319 ...
Protein Misfolding and Prions in Neurodegenerative Diseases. During neurodegenerative diseases biochemical changes in ... Breakthroughs in antemortem diagnosis of neurodegenerative diseases. Glenn C. Telling. PNAS first published October 30, 2019 ... Breakthroughs in antemortem diagnosis of neurodegenerative diseases Message Subject (Your Name) has sent you a message from ... However, the challenge underlying accurate detection of neurodegenerative diseases during their clinical phase is that specific ...
Learn how proteomics is being applied to neurodegenerative diseases in order to characterize the relationship existing between ... Table 1. Protein aggregates and related mitochondrial dysfunction in neurodegenerative disorders. Disease. Microscopic lesion. ... Mutations in specific genes have been identified as a causative event for many neurodegenerative diseases but the molecular ... Here, we review how proteomics has been applied to neurodegenerative diseases in order to characterize the relationship ...
"What kills neurons in neurodegenerative diseases?", a review series in an open access journal Authors: Todd E Golde and Leonard ... What have worm models told us about the mechanisms of neuronal dysfunction in human neurodegenerative diseases? The nematode ... Does neuroinflammation fan the flame in neurodegenerative diseases? While peripheral immune access to the central nervous ... Alzheimers disease: synaptic dysfunction and Aβ Synapse loss is an early and invariant feature of Alzheimers disease (AD) and ...
Neurodegener Dis (Source: Neurodegenerative Diseases). Source: Neurodegenerative Diseases - July 24, 2018 Category: Neurology ... Huntingtons Disease: Premotor Phase. Huntingtons disease (HD) is an incurable, neurodegenerative disease, which manifests via ... Neurodegener Dis 2017;17:I-IV (Source: Neurodegenerative Diseases). Source: Neurodegenerative Diseases - December 14, 2017 ... Neurodegener Dis 2018;18:1 -4 (Source: Neurodegenerative Diseases). Source: Neurodegenerative Diseases - January 16, 2018 ...
In a study of mouse models with Huntingtons disease, researchers found that eating at the same time every day improved motor ... Neurodegenerative disease: Restricting eating times may boost life quality. Published Wednesday 3 January 2018 Published Wed 3 ... Huntingtons disease is just one of many neurodegenerative disorders. Caused by an inherited mutation in a gene called HTT, ... The authors add, "Disturbances in the sleep/wake cycle are by now a well-established symptom of neurodegenerative diseases, and ...
Purchase Environmental Factors in Neurodegenerative Diseases, Volume 1 - 1st Edition. Print Book & E-Book. ISBN 9780128127643, ... Updates in this new volume include chapters on Air pollution and neurodegenerative diseases, Mercury and Parkinsons disease, ... Environmental Factors in Neurodegenerative Diseases, Volume 1 1st Edition. Write a review ... 5. Aluminum and Neurodegenerative Diseases. Stephen C. Bondy and Arezoo Campbell. 6. Manganese Neurodegeneration. David C. ...
... seemed a promising target in the treatment of brain diseases like multiple sclerosis or Alzheimers Disease. But clinical ... New impetus for treatment neurodegenerative diseases. University of Groningen. Journal. Proceedings of the National Academy of ... In the 1980s, it was discovered that in many neurodegenerative diseases it is TNF that makes the neurons die, says Eisel. ... A specific TNFR2 agonist could prevent cell death in neurodegenerative disease. We are now starting more trials in mouse ...
Neurodegenerative diseases as proteinopathies-driven immune disorders.. Ciccocioppo F1, Bologna G1, Ercolino E1, Pierdomenico L ... adaptive immunity; choroid plexus; immunotherapy; innate immunity; neurodegenerative diseases; neuroinflammation; ... New insights in the neurodegenerative field strongly suggest a role for the immune system in the pathophysiology of ... open new perspectives in the setting of specific immunotherapeutic strategies for the treatment of neurodegenerative diseases. ...
Implications for Disease. This ability of U1 to slide down just a single RNA base to recognize an atypical splice site might ... Although scientists have known about the gene mutation that causes the disease, they havent been able to explain why the ... The CSHL teams findings also have implications for studies aimed at uncovering and characterizing new disease-causing ... base-pair shifting explains why this mutation causes a severe disease. Krainer and Rocas results indicate that the correct ...
People who bear the genetic mutation for Huntingtons disease learn faster than healthy people. The more pronounced the ... The team has thus demonstrated for the first time that neurodegenerative diseases can go hand in hand with increased learning ... In Huntingtons disease, a short segment of a gene is repeated. The number of repetitions determines when the disease breaks ... Degenerative diseases of the nervous system are based on complex changes. A key mechanism is an increased release of the ...
A method of treating a neurodegenerative disease in a subject in need thereof comprises: (a) administering the subject an ... Huntingtons disease (HD) is one autosomal dominant polyglutamine-repeat disease. HD is a progressive, fatal neurodegenerative ... The method of claim 10, wherein said neurodegenerative disease is a polyglutamine repeat disease. 13. The method of claim 11, ... Neurodegenerative disease as used herein includes, for example, amyotrophic lateral sclerosis (or ALS), Parkinsons disease, ...
... we discuss the current status of genetic epidemiology of the most common neurodegenerative diseases: Alzheimer disease, ... Huntington disease, and prion diseases, with a particular focus on similarities and differences among these syndromes. ... Parkinson disease, Lewy body dementia, frontotemporal dementia, amyotrophic lateral sclerosis, ... that has allowed the elucidation of the molecular mechanisms underlying the etiology and pathogenesis of many neurodegenerative ...
Both SCA1 and Huntington s disease are members of a group of neurodegenerative disorders caused by a particular type of genetic ... symptoms and neurological damage caused by an inherited neurodegenerative disease that is similar to Huntington s disease (HD). ... to critical brain cells of mice with a disorder that mimics the human neurodegenerative disease spinocerebellar ataxia 1 (SCA1 ... progressive neurological diseases called polyglutamine-repeat diseases, which include HD and several spinocerebellar ataxias. ...
MSG and neurodegenerative disease. There is a relatively new area of research for which the food industry, to date, has had no ... These are studies which link MSG to neurodegenerative disease. Discovery that MSG caused lesions in specific areas of the ... At the present time, significant headway in the study of neurodegenerative disease is being made. Three EAA receptor subtypes ... Glutamic acid and aspartic acid are among the EAA that have great potential for involvement in neuro-degenerative disease. ...
Researchers are providing hope for some families with neurodegenerative conditions, after discovering a gene mutation ... Nutritional therapy could potentially treat childhood neurodegenerative disease. By Loren Grush Published August 01, 2013. Fox ... Childhood neurodegenerative diseases, while rare, can be devastating. Characterized by a progressive loss of neural function ... "Its a good example of a neurodegenerative disease no one expected to be treatable," Gleeson said. "Its a very sad condition. ...
While Alzheimers disease (AD) is the most frequent... ... nonenal pyrrole adducts in human neurodegenerative disease. J ... Phenotype Relationships in Neurodegenerative Diseases. Research and Perspectives in Alzheimers Disease. Springer, Berlin, ... While Alzheimers disease (AD) is the most frequent cause of dementia, other progressive neurodegenerative disorders can be ... Praticò D. (2005) Causes and Consequences of Oxidative Stress in Neurodegenerative Diseases. In: Cummings J.L., Poncet M., ...
... have found that a well-established anti-epileptic drug could also be used as a treatment for neurodegenerative diseases. ... possible-new-treatment-for-neurodegenerative-diseases-found/. More in Medicine & Health. * Simple surgery prevents strokes in ... Possible new treatment for neurodegenerative diseases found. University of Liverpool. Journal. Molecular Neurodegeneration. ... "Our research suggests that ethosuximide has potential for repurposing as a treatment for multiple neurodegenerative diseases ...
  • Neurodegenerative diseases are a heterogenous group of complex disorders linked by the degeneration of neurons in either the peripheral nervous system or the central nervous system . (wikipedia.org)
  • Nevertheless, our understanding of these disorders and the development of mechanistically designed therapeutics can still benefit from more rigorous use of the models and from generation of animals that more faithfully recapitulate human disease. (nature.com)
  • Huntington's disease is an hereditary neurodegenerative disease featured by alterations in movement, cognitive deficiency and psychiatric disorders resulting from the degeneration of neurons in the striatum nucleus of the brain. (news-medical.net)
  • Sleep quality tends to worsen with age and poor sleep is a modifiable risk factor for multiple disorders, including cardiovascular disease and cognitive impairment. (news-medical.net)
  • Most neurodegenerative disorders take decades to develop. (medscape.com)
  • Aggregation of the DNA/RNA-binding protein FUS (FUsed in Sarcoma) in the cytoplasm of nerve cells is characteristic of two devastating and incurable neurodegenerative disorders. (eurekalert.org)
  • Furthermore, accumulating evidence strongly supports the idea that other proteins implicated in the pathology of neurodegenerative disorders can form aggregates via phase separation," Dormann points out. (eurekalert.org)
  • The World Health Organization forecasts that within 2 decades neurodegenerative disorders will eclipse cancer to become the foremost cause of death in the developed world after cardiovascular disease. (pnas.org)
  • The involvement of tau in frontotemporal dementia with parkinsonism-17, Pick disease (PiD), progressive supranuclear palsy, argyrophilic grain disease, and corticobasal degeneration results in the blanket term of tauopathies to describe these disorders. (pnas.org)
  • Protein aggregates and related mitochondrial dysfunction in neurodegenerative disorders. (medscape.com)
  • Genetic loci identified in neurodegenerative disorders. (medscape.com)
  • Disease-Modifying Targets in Neurodegenerative Disorders: Paving the Way for Disease-Modifying Therapies examines specific neurodegenerative disorders in comprehensive chapters written by experts in the respective fields. (elsevier.com)
  • Prion diseases are disorders of protein conformation in which PrP C , the normal cellular conformer, is converted to an abnormal, protease-resistant conformer rPrP Sc . (biomedcentral.com)
  • α-Synuclein is a small protein that has special relevance for understanding Parkinson disease and related disorders. (biomedcentral.com)
  • Despite major advances in our understanding of the initiating factors that trigger many neurodegenerative disorders, to date, no novel disease-modifying therapies have been shown to provide significant benefit. (biomedcentral.com)
  • As promising leads are developed in one area, they can be tested in the other neurodegenerative disorders. (massgeneral.org)
  • We will briefly discuss how the clinical manifestations of common neurodegenerative disorders may be related with aberrant connectivity within large-scale neural networks. (medworm.com)
  • Eating meals at the same time each day may be an effective way to improve quality of life for individuals with neurodegenerative disorders, a new study suggests. (medicalnewstoday.com)
  • Researchers suggest that restricting eating times may benefit people with Huntington's disease and other neurodegenerative disorders. (medicalnewstoday.com)
  • Neurodegenerative disorders are conditions that involve the destruction of nerve cells. (medicalnewstoday.com)
  • Huntington's disease is just one of many neurodegenerative disorders. (medicalnewstoday.com)
  • The new study from Colwell and colleagues, however, suggests that a change in eating patterns may also help to improve the quality of life for individuals with Huntington's disease and other neurodegenerative disorders. (medicalnewstoday.com)
  • Neurodegenerative diseases as proteinopathies-driven immune disorders. (nih.gov)
  • In the pathophysiology of neurodegenerative disorders, the role of misfolded protein deposition leading to neurodegeneration has been primarily discussed. (nih.gov)
  • New insights in the neurodegenerative field strongly suggest a role for the immune system in the pathophysiology of neurodegenerative disorders. (nih.gov)
  • In fact, it has been the very knowledge gained from genetic studies that has allowed the elucidation of the molecular mechanisms underlying the etiology and pathogenesis of many neurodegenerative disorders. (jci.org)
  • Both SCA1 and Huntington s disease are members of a group of neurodegenerative disorders caused by a particular type of genetic flaw. (innovations-report.com)
  • Three EAA receptor subtypes that mediate excitotoxicity have been identified, drugs with anti-excitotoxic actions have been discovered, and evidence for the complicity of both exogenous and endogenous excitotoxins in neurodegenerative disorders has begun to unfold. (truthinlabeling.org)
  • 233. Coyle, J.T. Glutamate receptors and age-related neurodegenerative disorders. (truthinlabeling.org)
  • However, he is hopeful that AICAR will soon be developed into a drug by pharmaceutical companies, providing a model of treatment for other neurodegenerative disorders. (foxnews.com)
  • While Alzheimer's disease (AD) is the most frequent cause of dementia, other progressive neurodegenerative disorders can be responsible for it. (springer.com)
  • Although this syndrome is exceedingly rare, the mechanism implicated in it may help explain the origins of a variety of neurodegenerative disorders, such as Parkinson's and amyotrophic lateral sclerosis diseases, and even common depression and sleep disorders that are also hallmarks of the disorder, the researchers say. (redorbit.com)
  • These findings suggest that trafficking of specific cargoes inside brain cells may be a general problem in a variety of neurodegenerative diseases, depression, and other disorders. (redorbit.com)
  • The findings may also shed light on other neurodegenerative disorders, the researchers say. (redorbit.com)
  • What these clumps represent is not known, says co-author and neuropathologist Dennis Dickson, M.D. "But they are clearly a marker of the disease process in all of these disorders, suggesting a common process is perturbed," he says. (redorbit.com)
  • Neurodegenerative diseases are a heterogeneous group of complex disorders linked by the degeneration of neurons in either the peripheral nervous system or the central nervous system. (wikipedia.org)
  • Complex I deficiency, either specific or associated with other respiratory chain defects, has been identified in myopathies, encephalomyopathies and in three 'neurodegenerative' disorders: Parkinson's disease, dystonia and Leber's hereditary optic neuropathy. (nih.gov)
  • 1. Doty, K. R., Guillot-Sestier, M.-V. & Town, T. The role of the immune system in neurodegenerative disorders: Adaptive or maladaptive? (abcam.com)
  • Finally, through grants from ANR, LECMA/Vaincre Alzheimer, FRC and Neuratris, we assess whether reactive astrocytes impact molecular, cellular, functional and behavioral disease outcomes, in mouse models of Huntington's and Alzheimer's diseases (HD and AD), and more recently of demyelinating disorders. (cea.fr)
  • Alterations in cell surface protein signalling have been implicated in the pathogenesis of neurodegenerative disorders such as motor neuron diseases, PD and Alzheimer disease (AD) but also in diseases such depression, attention-deficit/hyperactivity disorder (ADHD) and schizophrenia. (plymouth.ac.uk)
  • Not surprisingly, neurodegenerative disorders that affect the brain on a cellular level may be evidenced in the retina. (healio.com)
  • Chapters cover metals in Alzheimer's Disease, Parkinson's Disease, Motor Neuron Disease, Autism and lysosomal storage disorders. (barnesandnoble.com)
  • His research has led to the development of first-in-class metal-drugs as a potential new therapeutic approach to treat motor neuron, Parkinson's and Alzheimer's diseases through the targeting of complementary biometal pathways in these disorders. (barnesandnoble.com)
  • NEW YORK (GenomeWeb) - Somatic mutations that arise during the development of the brain may underlie some neurodegenerative disorders like Alzheimer's and Parkinson's disease, according to a new study. (genomeweb.com)
  • The investigators propose using DaTscan in patients with REM sleep behavior disorder (RBD), mild cognitive impairment (MCI), Parkinson's disease (PD), dementia with Lewy bodies (DLB), Alzheimer's disease (AD), and other neurodegenerative syndromes and disorders, to test several hypotheses - some confirmatory, and some novel. (clinicaltrials.gov)
  • This Review summarizes how studies in Drosophila and mouse models accelerate our understanding of the links between circadian rhythm dysfunctions and age-related neurodegenerative disorders, especially Alzheimer's disease. (biologists.org)
  • Aberrant behavior by several proteins in the methyltransferase family is a possible cause of debilitating neurodegenerative disorders like Alzheimer's disease and Parkinson's disease. (sbir.gov)
  • Prior to its discovery, FXTAS was often misdiagnosed as other neurodegenerative disorders, such as Alzheimer's or Parkinson's diseases. (ucdavis.edu)
  • Investigators at The Neuro will generate cell lines lacking the proteins as a mechanism to validate the antibodies, and will then use their expertise in these disease domains, coupled with the Thermo Fisher-generated antibodies, to advance the understanding of these disorders. (mcgill.ca)
  • We believe that the discovery of bioactive agents that target pathways that are hit by multiple neurodegenerative conditions is the most viable approach in our current fight against brain disorders," Dr Cauchi stated. (prohealth.com)
  • Designer therapies are treatments tailored to a specific disease, and nowhere is the need greater for new therapies than in a group of nervous system disorders, known as "neurodegenerative diseases. (theconversation.com)
  • I decided to become a neurogeneticist - a geneticist who specializes in inherited neurological diseases - to focus on these disorders. (theconversation.com)
  • It turned out that prions and prion-like molecules are extremely relevant to some of the most common neurodegenerative disorders among humans. (brainblogger.com)
  • So, fast forwarding back to the present day-or June 28, 2018, at least-we see one company continuing the trend toward more focus on tau as news came out of Cantabio Pharmaceuticals Inc. that the preclinical-stage pharmaceutical company, which is working on therapeutics for AD, Parkinson's disease and related neurological disorders, had seen publication of a peer-reviewed article. (drugdiscoverynews.com)
  • AbstractPurpose of ReviewNeuropsychiatric syndromes (NPS) are common in neurodegenerative disorders (NDD). (medworm.com)
  • They hope that the methods may benefit patients suffering from neurodegenerative disorders one day. (medicalxpress.com)
  • This article will cover the epigenetics and treatment of Alzheimer's disease (AD), Huntington's disease (HD), and Parkinson's disease (PD). (wikipedia.org)
  • Huntington's Disease (HD) is a progressive neurodegenerative condition characterized by motor, cognitive, and psychiatric symptoms, and motor symptoms are often preceded by cognitive changes. (news-medical.net)
  • A novel machine-learning algorithm that uses genetic data from patient blood samples can significantly predict and explain the progression of neurodegenerative diseases such as Alzheimer's and Huntington's. (medscape.com)
  • The trial of nearly 2000 patients with late-onset Alzheimer's and Huntington's diseases shows that when applied to in vivo blood samples at baseline, the algorithm significantly predicted clinical deterioration and conversion to advanced disease stages. (medscape.com)
  • The investigators used data from 1969 patients with late-onset Alzheimer's and Huntington's disease from three large-scale databases, each of which was processed and analyzed independently. (medscape.com)
  • Alzheimer's, Parkinson's and Huntington's disease, and amyotrophic lateral sclerosis are the most relevant neurodegenerative syndromes worldwide. (medscape.com)
  • Protein variations detected in patients suffering from Alzheimer's, Parkinson's and Huntington's disease. (medscape.com)
  • The MassGeneral Institute for Neurodegenerative Disease (MIND) is a research center for Alzheimer's, ALS (Lou Gehrig's), Huntington's, Parkinson's and other neurodegenerative diseases. (massgeneral.org)
  • In a study conducted on mice with Huntington's disease , researchers discovered that restricting feeding time to the same 6-hour period every day for 3 months led to improvements in sleep quality and motor skills. (medicalnewstoday.com)
  • Caused by an inherited mutation in a gene called HTT , Huntington's disease is characterized by thinking problems and uncontrollable body movements. (medicalnewstoday.com)
  • It is estimated that around 30,000 people in the United States are living with Huntington's disease, and a further 200,000 people are at risk of developing the condition. (medicalnewstoday.com)
  • There are currently no treatments that can halt or reverse Huntington's disease, but there are medications that can help people with the condition to manage their symptoms. (medicalnewstoday.com)
  • To reach their findings, the scientists studied 6-month-old mouse models of Huntington's disease. (medicalnewstoday.com)
  • This region plays an important role in motor control, and it is subject to degeneration in people with Huntington's disease. (medicalnewstoday.com)
  • The authors add, "Disturbances in the sleep/wake cycle are by now a well-established symptom of neurodegenerative diseases, and here we show that we can treat the HD [Huntington's disease] symptoms by controlling the timing of food availability. (medicalnewstoday.com)
  • People who bear the genetic mutation for Huntington's disease learn faster than healthy people. (scienceblog.com)
  • In the current study, the researchers presented the same task to 29 subjects with the genetic mutation for Huntington's disease, who, however, did not yet show any symptoms. (scienceblog.com)
  • In Huntington's disease, a short segment of a gene is repeated. (scienceblog.com)
  • 4. The method of claim 4, wherein said polyglutamine repeat disease is selected from the group consisting of Huntington's disease, dentatorubral pallidoluysian atrophy, spinobulbar muscular atrophy, and spinocerebellar ataxia types 1, 2, 3, 6 and 7. (freepatentsonline.com)
  • Since these pathomechanisms are implicated to play a role in several neurodegenerative diseases, Cr supplementation as a neuroprotective strategy has received a lot of attention with several positive animal studies in models of Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS). (springer.com)
  • In the first part of this thesis, I combined modeling of the proteotoxicity of polyglutamine (as exemplified in Huntington's Disease) in Saccharomyces cerevisiae with microfluidics and automated microscopy. (mit.edu)
  • Researchers at Ruhr-Universit t Bochum and from Dortmund have found that people who are suffering from neurodegenerative diseases such as Huntington's disease have an ability to learn faster and the speed of their learning was directly linked to how pronounced their genetic mutation was, according to a new study published in the journal Current Biology. (medindia.net)
  • and a growing product line in the field of neurodegenerative diseases, such as Alzheimer's Disease, Parkinson's, Huntington's, and ALS. (cellsignal.com)
  • Dysregulation of glutathione homeostasis and alterations in glutathione-dependent enzyme activities are increasingly implicated in the induction and progression of neurodegenerative diseases, including Alzheimer's, Parkinson's and Huntington's diseases, amyotrophic lateral sclerosis, and Friedreich's ataxia. (unboundmedicine.com)
  • Newswise - CHAPEL HILL, NC - Scientists at the UNC School of Medicine have found a class of commonly used fungicides that produce gene expression changes similar to those in people with autism and neurodegenerative conditions, including Alzheimer's disease and Huntington's disease. (newswise.com)
  • Focusing on proteins such as Ataxin-1 known to be associated with NDDs such as Alzheimer's disease (AD), Parkinson's disease, Huntington's disease and spinocerebellar ataxia type 1, Hosp et al. (thermofisher.com)
  • The most studied neurodegenerative disease was Parkinson's disease, followed by dementia, amyotrophic lateral sclerosis (ALS), and Huntington's disease. (jneurosci.org)
  • Animal models of adult-onset neurodegenerative diseases have enhanced the understanding of the molecular pathogenesis of Alzheimer's disease, Parkinson's disease, frontotemporal dementia, and amyotrophic lateral sclerosis. (nature.com)
  • Here we review the current state of rodent models for Alzheimer's disease, Parkinson's disease, frontotemporal dementia, and amyotrophic lateral sclerosis. (nature.com)
  • An experimental medication that was recently shown to slow the progression of the neurodegenerative disease amyotrophic lateral sclerosis (ALS), or Lou Gehrig's disease, has now demonstrated the potential to also prolong patient survival. (news-medical.net)
  • Amyotrophic lateral sclerosis (ALS) is a motor neuron disease, which leads to progressive muscle weakness and ultimately to lethal paralysis, while frontotemporal dementia (FTD), the second most common form of dementia (after Alzheimer's) in those under 65. (eurekalert.org)
  • An accumulation of protein clumps is known to occur in a number of neurodegenerative diseases, including Alzheimer's disease , Parkinson's disease , and amyotrophic lateral sclerosis (ALS) - also known a Lou Gehrig's disease . (medicalnewstoday.com)
  • 5. The method of claim 1, wherein said neurodegenerative disease is a amyotrophic lateral sclerosis. (freepatentsonline.com)
  • In this review, we discuss the current status of genetic epidemiology of the most common neurodegenerative diseases: Alzheimer disease, Parkinson disease, Lewy body dementia, frontotemporal dementia, amyotrophic lateral sclerosis, Huntington disease, and prion diseases, with a particular focus on similarities and differences among these syndromes. (jci.org)
  • For example, in amyotrophic lateral sclerosis (ALS), a motor neuron disease also known as Lou Gehrig's disease, the molecular motors (for example, dynein, dynactin and kinesin) that drive transport from distant nerve terminals to the cell body may become defective. (redorbit.com)
  • These conditions include motor neuron diseases, such as amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease. (ucsfhealth.org)
  • Over the last 25 years my research has focused on the causative mechanisms, clinical manifestations, management, and treatment of motor neuron disease (amyotrophic lateral sclerosis), and other neurodegenerative diseases, particularly 'parkinson plus' syndromes, PSP and MSA. (sussex.ac.uk)
  • Lisa Landino studies the chemistry behind what she calls "the big three" neurodegenerative diseases: Parkinson's, amyotrophic lateral sclerosis and Alzheimer's. (wm.edu)
  • We conducted a retrospective mortality study of 17,321 PCB-exposed workers to determine whether mortality from Parkinson disease, dementia, and amyotrophic lateral sclerosis was elevated compared with the U.S. population. (cdc.gov)
  • We found no overall excess of Parkinson disease, amyotrophic lateral sclerosis, or dementia in the PCB-exposed cohort (standardized mortality ratios [SMRs]-1.40, 1.11, and 1.26, respectively, and number of deaths-14, 10, and 28 respectively). (cdc.gov)
  • The Montreal Neurological Institute (The Neuro) of McGill University, a world-leading institution focused on brain research and advanced patient care, and Thermo Fisher Scientific, the world leader in serving science, today announce an agreement designed to accelerate the understanding of neurological disease by focusing on about 30 proteins associated with Parkinson's disease, amyotrophic lateral sclerosis (ALS), hereditary spastic paraplegias, epileptic encephalopathies and ataxias, which are all devastating brain diseases. (mcgill.ca)
  • This cellular and molecular process diagram of neurodegeneration was created by integrating the mechanisms described in 89 neurodegenerative disease models published in the literature. (ebi.ac.uk)
  • This further hinders the understanding of the underlying dynamic molecular mechanisms involved in the pathogenesis of neurodegenerative conditions. (medscape.com)
  • Each chapter contains a summary of the disease management field, subsequently elaborating on the molecular mechanisms and promising new targets for disease-modifying therapies. (elsevier.com)
  • What have worm models told us about the mechanisms of neuronal dysfunction in human neurodegenerative diseases? (biomedcentral.com)
  • Extensive genetic, biochemical, and histological evidence has implicated the amyloid-β peptide (Aβ) in Alzheimer's disease pathogenesis, and several mechanisms have been suggested, such as metal binding, react. (biomedcentral.com)
  • While numerous hypotheses have been proposed to explain the molecular mechanisms underlying the pathogenesis of neurodegenerative diseases, the theory of oxidative stress has received considerable support. (biomedcentral.com)
  • Therefore, we have reviewed the pathogenic hypothesis in neurodegenerative pathologies, underling the links between the deposition of misfolded protein mechanisms and the immune activation. (nih.gov)
  • Neurodegenerative dementias: clinical features and pathological mechanisms. (springer.com)
  • In: Clark CM, Trojanowski JQ (eds) Neurodegenerative dementias: clinical features and pathological mechanisms. (springer.com)
  • The Hafezparast group use a cohort of mouse and induced pluripotent stem cell (iPS) model systems to gain insight into the molecular mechanisms of motor neuron disease caused by mutations in the components of axonal transport and RNA processing. (sussex.ac.uk)
  • Linda Van Eldik, Northwestern University, co-director of the Center for Drug Discovery and Chemical Biology, associate director of the Northwestern Alzheimer's Disease Center, studying mechanisms of glial activation and the potential of targeting chronic glial activation for AD therapeutics. (alzforum.org)
  • These immunolabeling methods further the investigation of molecular and neural circuit mechanisms of Alzheimer's disease. (cellsignal.com)
  • This book provides a clear explanation of different neurodegenerative diseases with new concepts of understand the etiology, pathological mechanisms, drug screening methodology and new therapeutic interventions. (intechopen.com)
  • The team uses zebrafish to study the neuronal mechanisms of these heavy metals in connection to neurodegenerative and neuromuscular diseases. (noldus.com)
  • Mechanisms of dysregulation of glutathione and/or glutathione-dependent enzymes are discussed that are implicated in pathogenesis of each neurodegenerative disease. (unboundmedicine.com)
  • Biometals in Neurodegenerative Diseases: Mechanisms and Therapeutics is an authoritative and timely resource bringing together the major findings in the field for ease of access to those working in the field or with an interest in metals and their role in brain function, disease, and as therapeutic targets. (barnesandnoble.com)
  • These diseases have different clinical manifestations due to different pathological mechanisms occurring in different parts of the brain. (frontiersin.org)
  • However, the ultimate result of the different disease mechanisms at the cellular level is loss of neurons and other cells in the brain. (frontiersin.org)
  • is one of the mechanisms by which hyperinflammation in brain diseases can be inhibited. (frontiersin.org)
  • With an aging population and lack of effective treatments for many of these conditions, it is critical to develop new tools and assays to advance research into the mechanisms of these diseases. (mcgill.ca)
  • Group's work is focused on mechanisms of neurodegeneration and subsequent loss of vision, particularly the early diagnosis and management of age-related neurodegenerative processes. (ucl.ac.uk)
  • While less lethal than mutations in the genome, such sublethal insults to cells might be associated with underlying mechanisms of several chronic diseases, including neurodegenerative disease. (mdpi.com)
  • Further investigations aimed at understanding the processing mechanisms related to oxidative RNA damage and its consequences may provide significant insights into the pathogenesis of neurodegenerative and other degenerative diseases and lead to better therapeutic strategies. (mdpi.com)
  • This JAX course will focus on the use of the innovative tools and methods, specifically in the laboratory mouse, for answering questions of neuron and circuit function and disease mechanisms during neurodegeneration. (jax.org)
  • See the Motor Neuron Fact Sheet for details regarding other motor neuron diseases. (wikipedia.org)
  • Conclusions: Despite some statistical instability of the results due to limitations in sample size, our study supports the role of CSF pNfH as a prognostic biomarker for motor neuron diseases presenting with UMN signs. (medworm.com)
  • Advanced Understanding of Neurodegenerative Diseases focuses on different types of diseases, including Alzheimer's disease, frontotemporal dementia, different tauopathies, Parkinson's disease, prion disease, motor neuron diseases such as multiple sclerosis and spinal muscular atrophy. (intechopen.com)
  • Neurodengenerative diseases of motor neurons can cause degeneration of motor neurons involved in voluntary muscle control such as muscle contraction and relaxation. (wikipedia.org)
  • Neurodegenerative diseases of sensory neurons can cause degeneration of sensory neurons involved in transmitting sensory information such as hearing and seeing . (wikipedia.org)
  • What kills neurons in neurodegenerative disease? (biomedcentral.com)
  • In the 1980s, it was discovered that in many neurodegenerative diseases it is TNF that makes the neurons die', says Eisel. (eurekalert.org)
  • A central nervous system disease that results in the progressive deterioration of function or structure of neurons. (yeastgenome.org)
  • In a subsequent study published in August 2008, the consortium reported that eight patients who died from the disease had substantial loss of neurons in the midbrain area known as the substantia nigra. (redorbit.com)
  • Neurodegenerative diseases - including Alzheimer's, Parkinson's, and multiple sclerosis - are marked by the progressive loss of brain cells called neurons. (mskcc.org)
  • Of particular interest, oxidative RNA damage can be demonstrated in vulnerable neurons early in disease, suggesting that RNA oxidation may actively contribute to the onset of the disease. (mdpi.com)
  • In all these diseases, a mutant protein that misfolds causes the degeneration and death of neurons. (theconversation.com)
  • As Emily Underwood wrote in a 2016 article in Science, "One of the telltale signs of Alzheimer's disease (AD) is sticky plaques of ß-amyloid protein, which form around neurons and are thought by a large number of scientists to bog down information processing and kill cells. (drugdiscoverynews.com)
  • Neurodegenerative diseases result from deterioration of neurons or their myelin sheath which over time will lead to dysfunction and disabilities resulting from this. (medicalxpress.com)
  • Inactivation of PI31 in these neurons was reminiscent of the severe behavioral and anatomical defects we see in some human neurodegenerative disease. (rockefeller.edu)
  • This landmark discovery found a correlation between the clumping of RNA-binding proteins long linked to neurodegenerative disease and the aggregates of protein found in the heart tissue of patients with RBM20 dilated cardiomyopathy. (news-medical.net)
  • This process - known as proteinopathy - occurs with different proteins in a variety of brain diseases and is believed to play a role in their progression. (medicalnewstoday.com)
  • During neurodegenerative diseases biochemical changes in particular proteins lead to their misfolding and accumulation in specific neuronal populations. (pnas.org)
  • Therefore, the hypothesis underlining the modulation of the innate and the adaptive immune system in the events linked to brain deposition of misfolded proteins could open new perspectives in the setting of specific immunotherapeutic strategies for the treatment of neurodegenerative diseases. (nih.gov)
  • Silencing the SCA1 gene with RNA interference inhibited the production of a neurotoxic protein, suggesting that this technology may also be helpful against other degenerative neurological diseases caused by neurotoxic proteins, such as Alzheimer s disease. (innovations-report.com)
  • Get to the root cause of neurodegeneration with a comprehensive range of neuroinflammation research tools to detect glia, aggregated proteins and proinflammatory mediators in Alzheimer's and Parkinson's diseases. (abcam.com)
  • The Caldecott lab is focussed on identifying and characterising novel human proteins involved in the repair of DNA breaks, with particular respect to neurological disease, and understanding how this knowledge can be exploited in the clinic. (sussex.ac.uk)
  • In particular, how the structure of the proteins involved in Alzheimer's disease may lead to neuronal dysfunction and cell death. (sussex.ac.uk)
  • Our cutting- edge antibody development technologies allow us to generate highly sensitive and specific antibodies to various amyloid-beta peptides, Tau phosphorylation sites, and other disease-research relevant proteins. (cellsignal.com)
  • From a more technical point of view, some chapters deal with the aggregation of prion proteins in prion diseases. (intechopen.com)
  • Presumably what happens is that alpha-synuclein prions in the MSA brain samples propagate by inducing the human alpha-synuclein proteins in the mice, which are prone to misfold, to take their particular aberrant shape Afterward, these mice's brains also showed buildups of alpha-synuclein in cells, and samples from these brains also caused disease in other mice. (scientificamerican.com)
  • Signalling network of GDNF/Ret with proteins encoded by genes mutated in some familial forms of Parkinson disease. (plymouth.ac.uk)
  • aggregates are associated with Alzheimer's disease, while TDP-43 , FUS and Ataxin-2 proteins are commonly found in MND patients. (tcd.ie)
  • The new discovery shows that Ataxin-2 concentrates several RNA-binding proteins used in the process of memory storing, but in doing so, it creates a biological environment that can help these proteins aggregate into disease-causing amyloids. (tcd.ie)
  • To accelerate the discovery of improved therapies for these diseases, we are developing screening kits to find drug molecules that correct the malfunctioning methyltransferase proteins. (sbir.gov)
  • As part of the study, Thermo Fisher will generate antibodies for proteins that investigators at The Neuro and other scientists have associated with particular neurological diseases. (mcgill.ca)
  • completed qAP-MS screens for five disease-specific proteins associated with the four NDDs. (thermofisher.com)
  • They compared the interactomics of wild type proteins with those found for the disease-associated variants. (thermofisher.com)
  • Furthermore, comparisons between wild type and disease-associated proteins showed that interactome maps generated were functionally relevant in AD. (thermofisher.com)
  • 2015) " Quantitative interaction proteomics of neurodegenerative disease proteins ," Cell Reports, 11(7) (pp.1134-46), doi: 10.1016/j.celrep.2015.04.030. (thermofisher.com)
  • The diseases are characterized by the accumulation of sticky proteins that cause damage to the nervous system. (prohealth.com)
  • Coincidentally, the accumulation of a misfolded protein in SCA7 is exactly the same type of molecular problem that occurs in Alzheimer's disease and Parkinson's disease, where misfolded proteins, albeit different ones, accumulate in the brains of patients. (theconversation.com)
  • Persistent failure to find any usual infectious agents led to the speculations that disease is transferred by proteins. (brainblogger.com)
  • Thanks to this new understanding of the role of prion-like proteins, a profound change in the way we think about the nature of neurodegenerative diseases has taken place in the recent years. (brainblogger.com)
  • And in a disease where sticky tangles of proteins seems to atrophy the brain and choke off cognition, one of the stickiest areas has been the issue of the amyloid protein vs. the tau protein. (drugdiscoverynews.com)
  • Oxidative stress could be a driving force in the spreading of aberrant proteins involved in Parkinson's disease. (medicalxpress.com)
  • Parkinson's disease, a neurodegenerative disorder that affects more than 6 million people worldwide, is caused by the buildup of alpha-synuclein proteins in the brain. (medicalxpress.com)
  • Peripheral nervous system (PNS) diseases may be further categorized by the type of nerve cell ( motor , sensory , or both) affected by the disorder. (wikipedia.org)
  • Parkinson's disease (PD) is the most common movement disorder and manifests as resting tremor, rigidity, bradykinesia, and postural instability. (biomedcentral.com)
  • A mutation in the CLCN6 gene is associated with a novel, particularly severe neurodegenerative disorder. (news-medical.net)
  • In a new study led by the Immune Systems Biology research group of the LIH Department of Infection and Immunity, researchers adopted a holistic machine-learning approach to elucidate how the interactions between neuronal mitochondria can serve as a powerful tool to distinguish nerve cells from Parkinson's patients from those belonging to healthy subjects, thereby providing new insights in the pathogenesis, diagnosis and treatment of this neurodegenerative disorder. (news-medical.net)
  • So a second aim of the study was to see if we can take a minimally invasive test such as a blood test and use it to give us an idea about which stage of disease patients are at, or how close they are to developing a particular disorder. (medscape.com)
  • This overview is ideal for neuroscientists, biomedical researchers, medical doctors, and caregivers, not only providing readers with a summary of the way patients are treated today, but also offering a glance at the future of neurodegenerative disorder treatment. (elsevier.com)
  • Davidson and her colleagues used a viral vector (a stripped-down virus) to deliver small fragments of genetic material (RNA) to critical brain cells of mice with a disorder that mimics the human neurodegenerative disease spinocerebellar ataxia 1 (SCA1). (innovations-report.com)
  • Now, researchers from the University of California, San Diego School of Medicine are providing hope for some families with neurodegenerative conditions, after discovering a gene mutation responsible for a very severe childhood disorder called pontocerebellar hypoplasia. (foxnews.com)
  • Disruptions in this railroad system have been seen in many neurodegenerative diseases, but these problems have been generally regarded as byproducts of the disorder rather than the cause, the researchers say. (redorbit.com)
  • We don't know if AICAR will work in mice or humans yet, but our work in cells definitely points in that direction," said co-author Vincent Cantagrel, PhD. "This rare disorder might be one of the first treatable neurodegenerative diseases in humans. (ucsd.edu)
  • Buildup in brain cells of the protein alpha-synuclein (dark spots) occurs in the neurodegenerative disorder Multiple System Atrophy (MSA). (scientificamerican.com)
  • Gaucher disease is an irreversible, inherited genetic metabolic disorder that results from not having enough glucocerebrosidase (GCase) - an enzyme that breaks down fatty chemicals called glucocerebrosides (GBA). (ucl.ac.uk)
  • X-linked adrenoleukodystrophy (X-ALD), which was first described in 1923, was viewed until 1976 as a rare and inexorably fatal neurodegenerative disorder that affected boys. (nih.gov)
  • The phenotypic variability of LBD is striking, as it can manifest as the well-known disorder of Parkinson's disease without (PD) and with dementia (PDD), as well as DLB, MCI, REM sleep behavior disorder (RBD), pure autonomic failure (PAF), and other syndromes. (clinicaltrials.gov)
  • FXTAS is a late-onset neurodegenerative disorder. (ucdavis.edu)
  • I became fascinated by diseases caused by these DNA repeats in 1991 when, as an M.D.-Ph.D. student, I discovered the first CAG repeat mutation responsible for a human disease called X-linked spinal and bulbar muscular atrophy, a rare neuromuscular disorder that affects only men, causing them to become weak and sometimes confined to a wheelchair. (theconversation.com)
  • Because SCA7 is a dominantly inherited genetic disease, each child of an affected SCA7 patient has a 50 percent chance of inheriting the mutant gene and developing the disorder. (theconversation.com)
  • Innate immune-mediated oxidative injury is a proposed driver of the neurodegeneration and demyelination characteristic of progressive neurodegenerative diseases like multiple sclerosis and Alzheimer's disease. (genengnews.com)
  • Spinocerebellar ataxia (SCA) is a progressive, neurodegenerative, genetic disease, which has no cure and treated only symptomatically. (medindia.net)
  • We hope that this strategy will be broadly applicable to halting progressive neurodegenerative disease. (theconversation.com)
  • Although treatments may help relieve some of the physical or mental symptoms associated with neurodegenerative diseases, there is currently no cure or way to slow disease progression. (nih.gov)
  • The identification of the etiology and additional factors contributing to the onset and progression of these diseases is of great importance in order to develop both preventive and therapeutic intervention. (medscape.com)
  • IMPRiND will construct this entire pipeline to examine the prion-like properties of α-synuclein and tau and test their tractability against disease progression. (europa.eu)
  • Other chapters discuss how hormones and health food supplements affect disease progression of neurodegenerative diseases. (intechopen.com)
  • Major contributors to initiation and progression of neurodegenerative diseases are considered, including oxidative stress, protein misfolding, and protein aggregation. (unboundmedicine.com)
  • Since such transmission occurs along neural connections rather than spatially or randomly, it is therefore emerging that the patterns of spread and progression of neurodegenerative diseases must consequently occur along and within the connectivity networks of the brain. (frontiersin.org)
  • Not only does this view provide a mechanistic basis that underlies and explains the highly stereotypical patterns seen in these diseases, it also enables quantitative and model-based exploration of progression in these diseases. (frontiersin.org)
  • So how is long-term memory formation and disease progression connected? (tcd.ie)
  • MMP-14 overexpression correlates with familial amyloidotic polyneuropathy disease and progression, as determined using mouse models and human samples. (biologists.org)
  • Characterizing protein-protein interactions (PPIs) could help researchers uncover pathways of disease progression, thereby aiding diagnostic and preventative strategies. (thermofisher.com)
  • Recent research findings demonstrate that the seeds of incorrectly folded beta-amyloid peptides can be transferred from one neuron to another, thus causing progression of the disease. (brainblogger.com)
  • Their density and pattern of distribution correlate closely with the stages of disease progression. (brainblogger.com)
  • As the company noted of the news, the aggregation of monomeric tau protein is linked to the onset and progression of Alzheimer's disease and other tauopathies, and this study and the scientific team's previous findings provide theoretical and experimental evidence for the ability of monomeric tau to be a receptor of small molecules designed to prevent the aggregation, which leads to toxicity and cell death. (drugdiscoverynews.com)
  • Modulation of neurotransmitter methylation is an emerging therapeutic strategy for the treatment of several neurodegenerative diseases, most notably Parkinson's and Alzheimer's diseases, and DNA and protein MTs are also being targeted for neurodegenerative diseases and cancers of the CNS. (sbir.gov)
  • Recently, oxidative RNA damage has been described in several neurodegenerative diseases including Alzheimer disease, Parkinson disease, dementia with Lewy bodies, and prion diseases. (mdpi.com)
  • Updates in this new volume include chapters on Air pollution and neurodegenerative diseases, Mercury and Parkinson's disease, Pesticides and PD: current evidence, Aluminum and neurodegeneration, Microglia and neurodegeneration, Dietary factors, Mitochondria in neurodegeneration, and Manganese and neurodegeneration. (elsevier.com)
  • And because Perry syndrome resembles many other neurodegenerative diseases, the findings suggest breakdowns along the cell's interior transportation grid may be a common mechanism underlying neurodegeneration. (redorbit.com)
  • One of the major roadblocks to medical progress in the field of neurodegeneration is the absence of models that fully recapitulate features of the human diseases. (idw-online.de)
  • The goal is to one day use this supplement to prevent or reverse the course of neurodegeneration in humans, and thus cure this disease," said Gleeson. (ucsd.edu)
  • Although the symptoms of some mild forms of Gaucher disease can be treated postnatally, more severe forms that cause early-onset, irreversible neurodegeneration are currently untreatable and are often fatal in infants. (ucl.ac.uk)
  • The implications of the published research, A pathoconnectome of early neurodegeneration: Network changes in retinal degeneration , extend far beyond eye diseases. (newswise.com)
  • The components of neurodegeneration we see in the eye seem to mimic those we see in the brain," explained the paper's lead author, Moran's Rebecca L. Pfeiffer, PhD. "So this pathoconnectome is allowing us to learn fundamental rules of how neurodegenerative diseases alter neural networks in general. (newswise.com)
  • The need to bring the research on biometals in neurodegeneration to the forefront of biomedical research is essential in order to understand neurodegenerative disease processes and develop effective therapeutics. (barnesandnoble.com)
  • Scientists have just discovered that a small region of a cellular protein that helps long-term memories form also drives the neurodegeneration seen in motor neuron disease (MND). (tcd.ie)
  • Here, we discuss the evidence implicating senescent cells in neurodegenerative diseases, the mechanistic contribution of these cells that may actively drive neurodegeneration, and how these cells or their effects may be targeted therapeutically. (jci.org)
  • The process of neurodegeneration is implicated in several degenerative diseases of the retina. (ucl.ac.uk)
  • Does neuroinflammation fan the flame in neurodegenerative diseases? (biomedcentral.com)
  • Our neuroinflammation antibody sampler panels allow you to try different antibody clones for key neuroinflammation disease targets to help you pick the right antibody for your research. (abcam.com)
  • This so-called neuroinflammation is commonly seen in autism and neurodegenerative conditions. (newswise.com)
  • Neurodegenerative diseases affect millions of people worldwide, and Alzheimer's disease and Parkinson's disease are the most common types. (nih.gov)
  • The beneficial role of MFG-E8 has been shown in cerebral ischemia (stroke), neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, and traumatic brain injury. (frontiersin.org)
  • Ruben J. Cauchi, PhD, and colleagues investigated the properties of brown seaweed and prickly pear in fruit fly models of Alzheimer's disease and Parkinson's disease. (prohealth.com)
  • The most relevant epigenetic modifications to treatment of neurodegenerative diseases are DNA methylation and histone protein modifications via methylation or acetylation. (wikipedia.org)
  • Ludwig-Maximilians-Universitaet (LMU) in Munich researchers have characterized the mechanism that initiates the pathological aggregation of the protein FUS, which plays a central role in two distinct neurodegenerative diseases. (eurekalert.org)
  • An enzyme that protects the brain against oxidative stress may also protect against the formation of protein clumps - a hallmark of Alzheimer's, Parkinson's, and other neurodegenerative diseases. (medicalnewstoday.com)
  • Alzheimer's disease (AD) is characterized by accumulation of extracellular plaques enriched with amyloid beta (Aβ) peptide derived from the amyloid precursor protein and intracellular neurofibrillary tangles composed of hyperphosphorylated microtubule-associated protein tau. (pnas.org)
  • Abnormal cytoplasmic accumulation of a normally soluble and unfolded protein called α-synuclein is the hallmark of diseases referred to as synucleinopathies. (pnas.org)
  • Here, we review how proteomics has been applied to neurodegenerative diseases in order to characterize the relationship existing between protein aggregation and mitochondrial alterations. (medscape.com)
  • The gene therapy also protected brain cells from the destruction normally caused by the disease and prevented the build-up of protein clumps within the cells. (innovations-report.com)
  • In these dominantly inherited diseases, a single mutated gene inherited from either parent produces a protein that is toxic to cells. (innovations-report.com)
  • In Alzheimer's disease, frontotemporal dementia and progressive supranuclear palsy, for instance, the "spikes," comprised of microtubule associated protein tau (MAPT), that normally stabilize and secure these rails tend to fall apart. (redorbit.com)
  • They also found a molecular signature of Perry syndrome "" "inclusions," or clumps, of a protein known as TDP-43 "" which is found in patients with frontotemporal dementia or with motor neuron disease. (redorbit.com)
  • Protein misfolding in neurodegenerative diseases: implications and strategies. (abcam.com)
  • The Serpell group are examining the structure of self-assembling peptides that are associated with protein misfolding diseases such as Alzheimer's disease. (sussex.ac.uk)
  • A common feature of neurodegenerative diseases is that they stem from problems with protein folding, but the underlying biology that leads to neuron death is not well understood. (mit.edu)
  • The idea that a protein could transmit disease was radical at the time but the work eventually earned Prusiner the 1997 Nobel Prize in Physiology or Medicine. (scientificamerican.com)
  • In 2013 a team in Prusiner's lab, including neuroscientist Kurt Giles, were trying to transmit Parkinson's disease to mice genetically engineered to produce a human protein involved in Parkinson's, alpha-synuclein, by injecting them with brain samples from deceased patients. (scientificamerican.com)
  • In recent years the ability of cell-to-cell transmission of protein species involved in almost all neurodegenerative diseases has been revealed from in vitro, animal models and human in vivo studies. (frontiersin.org)
  • His research has contributed to the understanding of copper interactions with the amyloid precursor protein (APP) and amyloid beta peptide in Alzheimer's disease, and more recently, the key role of copper in cell signaling and neuroinflammatory processes. (barnesandnoble.com)
  • A common feature of neurodegenerative diseases is the presence of specific protein aggregates in nerve cells, which accumulate and clump together - usually as protein fibres called amyloid filaments. (tcd.ie)
  • The Dictyostelium homologue of the Parkinson's disease-associated protein DJ-1 is located in the cytosol, and its loss causes cytopathological defects in endocytic and autophagic cell death pathways, but stimulates respiration by functionally normal mitochondrial respiratory complexes. (biologists.org)
  • Molecular pathological approaches may help clarify what makes each epicenter vulnerable to its targeting disease and how toxic protein species travel between networked brain structures," the authors write. (doctorslounge.com)
  • 1 Creating PPI networks and comparing wild type with disease-specific protein isoforms, the researchers hoped to gain insight on functional changes important in pathogenesis. (thermofisher.com)
  • They found that LRPPRC preferentially associated with the disease-specific form of Amyloid beta precursor protein (APPsw) found in early onset AD. (thermofisher.com)
  • Survival also improved in treated flies bred to express increased alpha-synuclein, a protein that accumulates in Parkinson's disease. (prohealth.com)
  • When the CAG sequence extends beyond 37 repeats, it causes disease because it causes the ataxin-7 protein to "misfold. (theconversation.com)
  • In looking at neurodegenerative diseases for this special focus section on neuroscience, it seemed fitting to check in not just on the consistently hot topic of Alzheimer's disease, but more specifically the issue of the tau protein and some of the recent insights and progress on the anti-tau front. (drugdiscoverynews.com)
  • The aggregation of the tau protein is a hallmark of Alzheimer's disease, but traditionally much of the energy and effort has gone toward focusing on ways to reduce the number of amyloid plaques. (drugdiscoverynews.com)
  • We are excited to publish further scientific evidence that establishes a structural biology basis for Cantabio's tau small-molecule pharmacological chaperone program, which aims to prevent and reduce aggregation of tau protein as a therapeutic strategy for Alzheimer's disease and other tauopathies such as concussion-related chronic traumatic encephalopathy," said Cantabio's CEO, Dr. Gergely Toth. (drugdiscoverynews.com)
  • Apolipoproten E (apoeE) is a major genetic risk factor for the development of Alzheimer's disease, yet the protein tends to be understudied as a potential druggable target for the mind-robbing neurodegenerative disease. (medicalxpress.com)
  • All neurodegenerative diseases have a common thread: the appearance of protein clumps in the brain such as amyloid-beta plaques in Alzheimer's disease and alpha synuclein aggregates in Parkinson's. (rockefeller.edu)
  • These diseases cause progressive deterioration of the neuron resulting in decreased signal transduction and in some cases even neuronal death. (wikipedia.org)
  • These diseases are characterized by chronic and progressive neuronal dysfunction, sometimes leading to behavioral abnormalities (as with PD), and, ultimately, neuronal death, resulting in dementia . (wikipedia.org)
  • These patients have what is described in medical textbooks as an untreatable disease, yet show mutations in a neuronal pathway that should be amenable to medication," said study co-author Naiara Akizu, PhD, a member of Gleeson's lab. (ucsd.edu)
  • Assemblies of tau and α-synuclein were shown to spread along interconnected neuronal networks suggesting broadly relevant therapeutic directions for Alzheimer's and Parkinson's disease. (europa.eu)
  • Such aggregates are believed to trigger processes that cause the neuronal death associated with these debilitating diseases. (tcd.ie)
  • Here, we focus on the pivotal role of MFG-E8 in protecting against neuronal diseases by promoting neurogenesis. (frontiersin.org)
  • A/T/N: An unbiased descriptive classification scheme for Alzheimer disease biomarkers. (nature.com)
  • LaFerla, F. M. & Green, K. N. Animal models of Alzheimer disease. (nature.com)
  • In Alzheimer disease brains, NMNAT2 levels are less than 50 percent of control levels, and we propose that enhancing NMNAT2 function may provide an effective therapeutic intervention to reserve cognitive function. (medicalnewstoday.com)
  • The following excerpt from the March 15, 2004 American Family Physician illustrates the relationship between glutamate and Alzheimer s disease. (truthinlabeling.org)
  • HIV patients with low white blood cell count due to immune suppression also showed reduced brain volume in brain regions like hippocampus and thalamus which are associated with various neurodegenerative diseases like Alzheimer s disease. (medindia.net)
  • That is, a person might have a gene that makes them more susceptible to a certain neurodegenerative disease, but whether, when, and how severely the person is affected depends on what environmental factors he or she is exposed to during life. (nih.gov)
  • Not surprisingly, there is a considerable lack of longitudinal gene expression data covering the complete evolution of neurodegenerative conditions. (medscape.com)
  • In the current study, Iturria-Medina and his team used these processes to analyze postmortem and in vivo gene expression neurodegenerative samples. (medscape.com)
  • Finally, the third dataset used data from 744 patients with blood-gene expression information from the Alzheimer's Disease Neuroimaging Initiative (ADNI). (medscape.com)
  • With an eye toward determining the molecular reconfigurations underlying neurodegenerative evolution, the investigators then reordered the gene expression patterns using a novel algorithm for detecting enriched trajectories in a diseased population relative to a background dataset of normal controls. (medscape.com)
  • Two molecular biologists at Cold Spring Harbor Laboratory have uncovered important new details about how a gene mutation causes a cellular editing error that results in a devastating disease called pontocerebellar hypoplasia (PCH). (redorbit.com)
  • Although scientists have known about the gene mutation that causes the disease, they haven't been able to explain why the mutation causes a defect in an essential cellular function called RNA splicing. (redorbit.com)
  • University of Iowa researchers have shown for the first time that gene therapy delivered to the brains of living mice can prevent the physical symptoms and neurological damage caused by an inherited neurodegenerative disease that is similar to Huntington s disease (HD). (innovations-report.com)
  • This is the first example of targeted gene silencing of a disease gene in the brains of live animals and it suggests that this approach may eventually be useful for human therapies," said senior study author Beverly Davidson, Ph.D., the Roy J. Carver Chair in Internal Medicine and UI professor of internal medicine, physiology and biophysics, and neurology. (innovations-report.com)
  • The genetic material suppresses the disease-causing SCA1 gene in a process known as RNA interference. (innovations-report.com)
  • In contrast, mice with the SCA1 disease gene that were not treated developed movement problems and lost brain cells in a manner similar to humans with this condition. (innovations-report.com)
  • Thus, a successful therapy must remove or suppress the disease-gene rather than simply add a corrected version. (innovations-report.com)
  • In addition to the finding that RNA interference inhibited gene expression to such an extent that it protected the animals against the disease, another important finding was that RNA interference in and of itself does not appear to be toxic to normal brain cells. (innovations-report.com)
  • Furthermore, the study revealed that specific properties of different gene therapy vectors can be used to target those cells that are most involved in causing the disease symptoms. (innovations-report.com)
  • Davidson is optimistic about the potential for using RNA interference gene therapy to treat neurological diseases like HD and spinocerebellar ataxias in humans. (innovations-report.com)
  • In some forms of Parkinson's disease, growing evidence indicates that the cargoes being trafficked are also misdirected by faulty signaling, due to pathogenic mutations in the leucine-rich repeat kinase 2(LRRK2) gene, Dr. Farrer says. (redorbit.com)
  • Fetal gene therapy for neurodegenerative disease of infants. (nih.gov)
  • For inherited genetic diseases, fetal gene therapy offers the potential of prophylaxis against early, irreversible and lethal pathological change. (nih.gov)
  • Researchers at the University of California, San Diego School of Medicine have identified the gene mutation responsible for a particularly severe form of pontocerebellar hypoplasia, a currently incurable neurodegenerative disease affecting children. (ucsd.edu)
  • A mutation in the gene can result in pontocerebellar hyplasia, a neurodegenerative disease afflicting children. (ucsd.edu)
  • Samples from the brains of mice with two copies of the gene also caused disease when injected into other mice, but only after the longer, 10-month period. (scientificamerican.com)
  • A fatal neurodegenerative condition known as Gaucher disease can be prevented in mice following fetal gene therapy, finds a new study led by UCL, the KK Women's and Children's Hospital and National University Health System in Singapore. (ucl.ac.uk)
  • The study, published today in Nature Medicine, highlights the potential of fetal gene therapy to prevent and cure neonatal lethal neurodegenerative diseases in humans in utero. (ucl.ac.uk)
  • Mutations in the GBA gene, which encodes the GCase enzyme that is deficient in Gaucher disease, are also a risk factor for Parkinson's disease. (ucl.ac.uk)
  • We found that the mice who received an injection of adeno-associated virus (AAV) vector were more able to break down fatty chemicals and re-express the gene encoding an enzyme that is deficient in Gaucher disease," said Dr Simon Waddingdon (UCL Institute for Women's Health). (ucl.ac.uk)
  • This new approach will bring hope, not only for Gaucher disease, but also for other inborn errors of metabolism that can potentially be treated using fetal gene therapy," said Associate Professor Jerry Chan, Senior Consultant, Department of Reproductive Medicine, KK Women's and Children's Hospital. (ucl.ac.uk)
  • The team, which also involved scientists from King's College London, Imperial College London, the University of Oxford and an international team of researchers, are now engaged with Apollo Therapeutics in developing gene therapy for Gaucher disease. (ucl.ac.uk)
  • Transcriptome profiling of Friedreich's ataxia fibroblasts by RNA sequencing reveals that this peripheral tissue can be used as a disease model for gene expression biomarker discovery. (biologists.org)
  • A new UNC School of Medicine study shows how chemicals designed to protect crops can cause gene expression changes in mouse brain cells that look strikingly similar to changes in the brains of people with autism and Alzheimer's disease. (newswise.com)
  • The disease occurs when a short section of DNA that encodes ataxin-7 gene is erroneously repeated - like a word in a book printed two or three times. (theconversation.com)
  • and mutations in the PI31 gene itself have been linked to Alzheimer's disease. (rockefeller.edu)
  • Despite this rapid increase in our understanding of biometals in brain disease, the fields of biomedicine and neuroscience have often overlooked this information. (barnesandnoble.com)
  • Lead authored by Cantabio's CEO Dr. Gergely Toth, along with collaborators at the Hungarian Academy of Sciences and German Center for Neurodegenerative Diseases (DZNE), the work appeared in the journal ACS Chemical Neuroscience . (drugdiscoverynews.com)
  • Work on neurodegenerative disease was not a major focus in neurology or neuroscience 40 years ago. (jneurosci.org)
  • This five day course is focused on the use of the mouse in neuroscience studies, particularly neurodegenerative disease. (jax.org)
  • CURE-ND, or Catalysing a United Response in Europe to Neurodegenerative Diseases, is a new alliance between the German Center for Neurodegenerative Diseases (DZNE), the Paris Brain Institute (ICM), Mission Lucidity (ML) and the UK Dementia Research Institute (UK DRI). (idw-online.de)
  • The German Center for Neurodegenerative Diseases (German: Deutsches Zentrum für Neurodegenerative Erkrankungen, (DZNE)) is an interdisciplinary research institution that investigates neurodegenerative disease in all its facets. (wikipedia.org)
  • This is the result of lab studies by researchers of the German Center for Neurodegenerative Diseases (DZNE). (medicalxpress.com)
  • Neurodegenerative diseases occur when nerve cells in the brain or peripheral nervous system lose function over time and ultimately die. (nih.gov)
  • Neurodegenerative diseases of the central nervous system can affect the brain and/or spinal cord . (wikipedia.org)
  • Brain disease such as Alzheimer's and Parkinson's affect an estimated one in six Americans and are increasing in incidence as the population ages. (scientificamerican.com)
  • Furthermore, the technique also allowed for discovery of genes and molecular pathways in peripheral tissues and brain tissues that are highly predictive of disease evolution. (medscape.com)
  • That's very important because there has been a lot of emphasis on finding brain biomarkers of neurodegenerative diseases like Alzheimer's. (medscape.com)
  • Non-invasive brain stimulation (NIBS) is emerging as a promising rehabilitation tool for a number of neurodegenerative diseases. (medworm.com)
  • Twenty years ago, tumor necrosis factor (TNF) seemed a promising target in the treatment of brain diseases like multiple sclerosis or Alzheimer's Disease. (eurekalert.org)
  • Likewise, it was study of the brain that suggested that a group of these neurotransmitters, specifically the excitatory amino acids (EAA), possess properties that very likely play an important role in the development of certain neurodegenerative diseases(172, 229-236). (truthinlabeling.org)
  • Traditional views have claimed that oxidative-mediated brain injury in these diseases is merely the result of the neurodegenerative processes. (springer.com)
  • These brain scans highlight the deficits in brain areas that are also vulnerable to age-related neurodegenerative diseases. (medindia.net)
  • HIV-related pathological processes and age like inflammation and blood brain barrier impairment can accelerate age-related neurodegenerative processes. (medindia.net)
  • This study will test whether the drug 11C-ER176 can show brain inflammation on positron emission tomography (PET) scans in people with a neurodegenerative disease, such as Alzheimer's disease or frontotemporal dementia, compared to healthy individuals. (nih.gov)
  • A chronic inflammatory environment in the brain is a hallmark of neurodegenerative diseases such as Alzheimer's and Parkinson's 4,5 . (abcam.com)
  • Assess whether reactive astrocytes can be monitored with non-invasive brain imaging techniques to serve as biomarkers for brain diseases (see part 3). (cea.fr)
  • Evaluate therapeutic efficacy of targeting reactive astrocytes in brain diseases (see part 4). (cea.fr)
  • Reactive astrocytes appear under pathological conditions, and thus could be used as biomarkers for brain diseases. (cea.fr)
  • Researchers have known for some time that a common feature of many neurodegenerative diseases is the heightened activity of helper cells in the brain called macrophages. (mskcc.org)
  • In this webinar the speaker will discuss the use of Alzheimer's disease transgenic mouse models to evaluate pathological features by both traditional immunohistochemistry and new tissue processing methods that enable whole- brain imaging. (cellsignal.com)
  • Neither a sample from a disease-free brain nor samples from Parkinson's patients, had these effects. (scientificamerican.com)
  • We solicit submissions concerned with mathematical, graph theoretic or other network-based models that can capture or inform the spread of neurodegenerative pathologies in a whole-brain and quantitative fashion. (frontiersin.org)
  • Cells with features of senescence have been detected in the context of brain aging and neurodegenerative disease, suggesting that they may also promote dysfunction. (jci.org)
  • We propose further investigation of the molecular pathways for MFG-E8 signaling in NSPC and effective strategies for MFG-E8 delivery across the blood-brain barrier, which will help develop MFG-E8 as a future drug candidate for the bedside management of neurodegenerative diseases. (frontiersin.org)
  • Neurodegenerative diseases may be characterized by specific regions of the brain that are critical network epicenters, with disease-related vulnerability associated with shorter paths to the epicenter and greater total connectional flow, according to a study published in the March 22 issue of Neuron . (doctorslounge.com)
  • FRIDAY, March 23 (HealthDay News) -- Neurodegenerative diseases may be characterized by specific regions of the brain that are critical network epicenters, with disease-related vulnerability associated with shorter paths to the epicenter and greater total connectional flow, according to a study published in the March 22 issue of Neuron . (doctorslounge.com)
  • To investigate how intrinsic connectivity in health predicts regional vulnerability to neurodegenerative disease, Juan Zhou, Ph.D., from the University of California in San Francisco, and colleagues used task-free functional magnetic resonance imaging to identify the healthy intrinsic connectivity patterns seeded by brain regions vulnerable to five neurodegenerative diseases (Alzheimer's disease, behavioral variant frontotemporal dementia, semantic dementia, progressive nonfluent aphasia, and corticobasal syndrome). (doctorslounge.com)
  • The researchers also found that these chemicals stimulated the production of free radicals - particles that can damage the basic building blocks of cells and that have been implicated in a number of brain diseases. (newswise.com)
  • Neurodegenerative diseases like Alzheimer's and Parkinson's are partly attributable to brain inflammation. (bio-medicine.org)
  • Spinocerebellar ataxia type 7 (SCA7) is one such disease in which nerves in different parts of the brain, including the eye, degenerate, which leads to blindness and difficulty walking, speaking and balancing. (theconversation.com)
  • Brain cells get lost and some regions of cerebral cortex shrink in size as the disease progresses. (brainblogger.com)
  • Classical hallmarks of Alzheimer's disease - amyloid plaques and neurofibrillary tangles - are typically observed in the brain of patients with this condition. (brainblogger.com)
  • Neurodegenerative disease (Greek νέυρο-, néuro- , "nerval" and Latin dēgenerāre , "to decline" or "to worsen") is a condition in which cells of the brain and spinal cord are lost. (medicalxpress.com)
  • Cells of the brain and spinal cord are not readily regenerated en masse , so excessive damage can be devastating. (medicalxpress.com)
  • Degenerative nerve diseases can be serious or life-threatening. (medlineplus.gov)
  • Degenerative diseases of the nervous system are based on complex changes. (scienceblog.com)
  • Glutamic acid and aspartic acid are among the EAA that have great potential for involvement in neuro -degenerative disease. (truthinlabeling.org)
  • Neuro-degenerative diseases including Autism are characterized by the proliferation of cells from mutations of the neuron's DNA sequence. (prlog.org)
  • Indeed, oxidative DNA damage has been detected in tissues of schizophrenic patients, the consequence of which the deficient repair of DNA that contributes to degenerative disease. (prlog.org)
  • The source of neuro-degenerative diseases from the newborn to the elderly is the DNA damage from UV radiation created by simple QED from diverse NPs that enter the body in vaccines and GM food. (prlog.org)
  • Along with a general decline in overall health, most chronic degenerative human diseases are inherently associated with increasing age. (jci.org)
  • A specific TNFR2 agonist could prevent cell death in neurodegenerative disease. (eurekalert.org)
  • Researchers worldwide are making a strong effort to understand neurodegenerative diseases pathogenesis, which is essential to develop efficient treatments against these incurable diseases. (news-medical.net)
  • This new discovery by researchers at Umeå University, Sweden, in collaboration with researchers in Croatia and Lithuania, provides renewed hope for novel treatments of, for instance, Alzheimer's and Parkinson's disease in the long run. (news-medical.net)
  • Overall, the researchers believe their finding could pave the way for new treatments for a number of neurodegenerative diseases. (medicalnewstoday.com)
  • At MIND, a team of world-renowned experts and young, innovative researchers are working in a center that allows them to collaborate, strategize, and share technology to move from basic research to treatment for these diseases. (massgeneral.org)
  • Researchers at the University of Liverpool have found that a well-established anti-epileptic drug could also be used as a treatment for neurodegenerative diseases. (eurekalert.org)
  • Researchers from the University's Institute of Translational Medicine have found that the anti-epileptic drug ethosuximide has protective effects in certain neurodegenerative disease models. (eurekalert.org)
  • The researchers tested their AICAR-based treatment in genetic models of the disease and in human cells. (ucsd.edu)
  • For the thousands of people diagnosed each year with a neurodegenerative disease, a sobering fact compounds their suffering: Researchers do not know what causes these diseases, let alone what can be done to treat them in most cases. (mskcc.org)
  • In the 1960s researchers led by Carleton Gajdusek at the National Institutes of Health transmitted kuru, a rare neurodegenerative disease found in Papua New Guinea, and Creutzfeldt-Jakob disease (CJD), a rare human dementia, to chimpanzees by injecting samples from victims' brains directly into those of chimps. (scientificamerican.com)
  • In the study, published in Nature Communications today, researchers led by a team at the University of Cambridge in the UK found that samples from more than half of 54 brains from patients and controls harbored somatic mutations, some of which may account for their disease. (genomeweb.com)
  • To examine the interactome data in association with human disease, the researchers conducted a genome-wide association study for AD. (thermofisher.com)
  • The researchers acknowledge that the initial interactome d ata come f rom overexpression studies in a human cell line and therefore may not be completely applicable to in vivo disease development. (thermofisher.com)
  • Alzheimer's has already proven to be a particularly complex and challenging disease for life-sciences researchers and pharma/biotech companies. (drugdiscoverynews.com)
  • However, the challenge underlying accurate detection of neurodegenerative diseases during their clinical phase is that specific biomarkers are not present at high enough concentrations for routine detection in accessible specimens. (pnas.org)
  • Clark CM (2000) Clinical manifestations and diagnostic evaluation of patients with Alzheimer's disease. (springer.com)
  • In a study published in 2007, Dr. Wszolek, along with Swiss neurologist and visiting fellow Christian Wider, M.D., summarized the clinical features of the disease, which include early-onset parkinsonism (stiffness, slowness and rigidity), depression, severe weight loss, and increasing difficulty in breathing. (redorbit.com)
  • Taken together, the use of Cr supplementation has so far proved disappointing in clinical studies with a number of symptomatic neurodegenerative diseases. (springer.com)
  • At any given time, UCSF is conducting more than 1,500 clinical trials to better understand disease and evaluate new treatments. (ucsfhealth.org)
  • Alzheimer's clinical trials thus far have failed to reach primary endpoints, and the clinical and research community recognizes that a better understanding of the pathophysiology and treating at earlier stages of these diseases are the roads forward for effective therapeutic intervention. (healio.com)
  • A robust, relatively low-cost, and patient-friendly biomarker for early diagnosis of neurodegenerative diseases, perhaps even before clinical symptomatology, is therefore currently a large unmet need. (healio.com)
  • He has published over 120 original research papers, reviews and book chapters, is Associate Editor for Neurochemistry International and co-founded a start-up biotech company (Procypra Therapeutics) that has delivered a novel copper-based metal-drug to clinical trials for motor neuron disease. (barnesandnoble.com)
  • Such use will provide new data on the potential clinical and research utility of DaTscan in neurodegenerative diseases. (clinicaltrials.gov)
  • Most high-profile clinical trials for neurodegenerative diseases have led to inefficacious results, suggesting that novel approaches to treating these pathologies are needed. (jci.org)
  • These are to be achieved using novel non-invasive techniques to assess structural and functional changes in different models of disease and their treatment, with a view to offering quick and effective translation to the clinical arena. (ucl.ac.uk)
  • The clinical research study is not designed to exclusively test toxicity or disease pathophysiology in healthy individuals. (cms.gov)
  • are confident that quantitative interaction proteomics can aid clinical research in determining the role played by disease-specific variants in pathogenesis of NDD phenotypes. (thermofisher.com)
  • It has been asserted that identification of such beta amyloid plaque can inform the clinical management of patients with cognitive impairment who are being evaluated for possible Alzheimer's disease or other causes cognitive decline. (cms.gov)
  • Neurology, in 1969, was focused on diagnosis of the disease, primarily by physical examination. (jneurosci.org)
  • A review of mechanistic models on neurodegenerative disease processes was published in CPT:PSP. (ebi.ac.uk)
  • Simultaneously, single-cell, multi-omics and optogenetics technologies now allow longitudinal, molecular and functional analysis of human disease processes in these models at high resolution. (idw-online.de)
  • 11C-ER176 is a radioligand, a radioactive substance that is injected into the body to help diagnosis diseases or study processes within the body. (nih.gov)
  • He investigates the cellular pathology of neurodegenerative diseases, and has a strong focus on the role of biometals in neurodegenerative processes, and as novel targets for neurotherapeutics. (barnesandnoble.com)
  • The authors review the α-synuclein structural, biophysical and biochemical properties that influence relevant mitochondrial physiological processes such as fusion-fission, transport and clearance, and propose that α-synuclein contributes to the mitochondrial defects that are associated with Parkinson's disease. (biologists.org)
  • This unique structure of MFG-E8 enables it to mitigate several pathological processes involved in neurodegenerative diseases. (frontiersin.org)
  • The Institute's mission is to accelerate research discoveries that will lead to treatment and cures for neurodegenerative diseases. (massgeneral.org)
  • The ultimate goal of JPND is to find cures for neurodegenerative diseases and to enable early diagnosis for early targeted treatments. (sfi.ie)
  • The Centers for Medicare & Medicaid Services (CMS) has convened this meeting for the panel to review available evidence and hear public testimony on the use of beta amyloid PET imaging for the management of dementia and neurodegenerative disease. (cms.gov)
  • health care professionals can take it as a reference book, even patients' families, relatives and friends can take it as a good basis to understand neurodegenerative diseases. (intechopen.com)
  • Professor of Cellular and Molecular Physiology, Alan Morgan, said: "Incidence of these diseases is on the rise due to our increasingly ageing population, yet there is a lack of effective therapies to treat them. (eurekalert.org)
  • By blocking a particular family of enzymes called GCK-IV, it may be possible to develop new therapies for treating neurodegenerative diseases like glaucoma and Alzheimer's disease. (medindia.net)
  • The ultimate goal is to identify how we might develop new therapies based on preventing or interfering with the network rewiring prompted by disease. (newswise.com)
  • A reliable, precise, and easily accessible biomarker for neurodegenerative diseases is crucial to identify in order to diagnose and monitor patients, and also to help possibly develop new therapies to treat these devastating diseases. (healio.com)
  • This partnership with Thermo Fisher Scientific is the most recent open science agreement The Neuro has forged to develop effective therapies for neurological disease, including agreements with the Structural Genomics Consortium, Takeda Pharmaceutical Company Ltd., the Centre for Drug Research and Development (CDRD), and Merck. (mcgill.ca)
  • and "Why don't we have effective therapies for these devastating diseases? (jneurosci.org)
  • Epilepsy, migraine, and Parkinson's disease (PD) had the most effective therapies. (jneurosci.org)
  • These substances can provide a breakthrough in the treatment of neurodegenerative pathologies. (news-medical.net)
  • Next to that, the increased uncontrolled movements of the larvae exposed to high levels of aluminum are considered important, because uncontrolled movements are typical of neurologic pathologies like Parkinson's disease. (noldus.com)
  • There is expected to be a considerable increase in the incidence of neurodegenerativediseases such as mild cognitive impairment (MCI), Alzheimer's disease (AD), and Parkinson's disease (PD). (healio.com)
  • More than five million Americans are living with Alzheimer's disease, and at least 500,000 Americans live with Parkinson's disease, although some estimates are much higher. (nih.gov)
  • In the second part of this thesis, I quantitatively and systematically studied the toxicity of a-synuclein, which is implicated in the synucleinopathy family of diseases, including Parkinson's Disease. (mit.edu)
  • In this installment of Practical Retina, Dilraj S. Grewal, MD , and Sharon Fekrat, MD , both of Duke Eye Center, provide a timely update on the use of OCT imaging in the detection of neurodegenerative disease, including Alzheimer's, dementia, and Parkinson's disease. (healio.com)
  • In these patients, DaTscan may be used to help differentiate essential tremor from tremor due to Parkinsonian syndromes (such as idiopathic Parkinson's disease, multiple system atrophy and progressive supranuclear palsy). (clinicaltrials.gov)
  • Mitochondrial dynamics in Parkinson's disease: a role for α-synuclein? (biologists.org)
  • As part of our pledge to provide validated models to better recapitulate in vivo systems, ATCC has created neural progenitor cells (NPCs) from normal and Parkinson's disease-derived induced pluripotent stem cells. (atcc.org)
  • In this webinar we will focus on the Parkinson's disease-derived NPCs, detailing their neural biomarker expression profiles during dopaminergic neuron differentiation and validating their use in toxicological studies. (atcc.org)
  • The pesticide rotenone was previously implicated in Parkinson's disease through replicated animal experiments and through human epidemiological studies. (newswise.com)
  • A separate 2015 UNC study found that Parkinson's disease is much more common in older adults with autism than in older adults without autism. (newswise.com)
  • Many of these diseases are common and well-known, such as Alzheimer's or Parkinson's disease. (theconversation.com)
  • Alan Alda has Parkinson's disease. (theconversation.com)
  • Prions have offered surprising and unexpected explanations to the development of such conditions as Alzheimer's disease, Parkinson's disease, motor neuron disease and many related syndromes. (brainblogger.com)
  • We collaborated with the Parkinson's Institute to understand the molecular basis for Parkinson's disease. (thermofisher.com)
  • Read about how we used stem cells to develop cellular models for understanding Parkinson's disease. (thermofisher.com)
  • With one exception -pimavanserin is approved for the treatment of hallucinations and delusions in Parkinson's disease-there are no drugs approved by the FDA for treatment of NPS in NDD. (medworm.com)
  • Trials show that atypical antipsychotics reduce psychosis in AD and in Parkinson's disease, although side effect concerns hav e constrained their use. (medworm.com)
  • Antidepressants show benefit in treatment of Parkinson's disease with depression. (medworm.com)
  • Not only is α-synuclein found in Lewy bodies characteristic of Parkinson disease, but also m. (biomedcentral.com)
  • Ahlskog JE (2007) I can't get no satisfaction: still no neuroprotection for Parkinson disease. (springer.com)
  • Loss of dopamine is the hallmark of Parkinson disease, a neurodegenerative disease. (cdc.gov)
  • Eve Topf and USA National Parkinson Foundation Centers of Excellence for Neurodegenerative Diseases Research and Department of Pharmacology, Technion-Faculty of Medicine, Haifa, Israel. (cyclingforums.com)
  • It might offer potential treatments for this specific condition and offer hope for other neurodegenerative treatments. (foxnews.com)
  • Neurodegenerative diseases such as Alzheimer's and Parkinson's are not well understood, and there are few effective treatments. (mskcc.org)
  • The pathoconnectome can be used to identify potential new treatments for neurodegenerative diseases, including epilepsy and Alzheimer's, by showing how they rewire neural systems. (newswise.com)
  • Scientists are challenged by the lack of advanced biological models of the nervous system to produce meaningful results that lead to better, more precise treatments for neurodegenerative diseases. (atcc.org)
  • Neurodegenerative diseases are devastating medical conditions with no effective treatments. (frontiersin.org)
  • Effective treatment of these diseases is often prevented by lack of understanding of the underlying molecular and genetic pathology. (wikipedia.org)
  • [1] [2] Though some sensory neuron diseases are recognized as neurodegenerative, epigenetic factors have not yet been clarified in the molecular pathology. (wikipedia.org)
  • Research into the molecular and cellular basis of motor neuron disease is led by Hafezparast Group. (sussex.ac.uk)
  • Our research suggests that ethosuximide has potential for repurposing as a treatment for multiple neurodegenerative diseases and provides a platform from which new medicines could be developed. (eurekalert.org)
  • This creates a critical need to improve our understanding of what causes neurodegenerative diseases and develop new approaches for treatment and prevention. (nih.gov)
  • If the therapeutic approach can be extended to humans, it may provide a treatment for a group of incurable, progressive neurological diseases called polyglutamine-repeat diseases, which include HD and several spinocerebellar ataxias. (innovations-report.com)
  • Memantine ( Namenda ) is an N-methyl-d-aspartate (NMDA) receptor blocker indicated for the treatment of moderate to severe Alzheimer's disease (AD). (truthinlabeling.org)
  • The supplement was tested in genetic models of the disease, as well as human cells in vitro, and the treatment successfully rescued the cells from degeneration and death. (foxnews.com)
  • The results challenge the conventional thinking and open up potential avenues for the diagnosis and treatment of neurodegenerative diseases. (mskcc.org)
  • Age-associated cognitive impairments and neurodegenerative diseases, such as Parkinson's and Alzheimer's diseases, are potentially debilitating conditions that lack viable options for treatment, resulting in a tremendous economic and societal cost. (jci.org)
  • We'll now be examining whether the substances that inhibit the caspases can be candidates for useful drugs in the treatment of certain neurological diseases," says Dr Joseph. (bio-medicine.org)
  • There are other reasons to suspect that the lab's findings could inform the treatment of neurodegenerative diseases. (rockefeller.edu)
  • Under pathological conditions, such as neurodegenerative diseases, astrocytes become reactive. (cea.fr)
  • Alzheimer's disease presents stereotypical pathological features including the abundance of amyloid deposition and tauopathy. (cellsignal.com)
  • Various mouse lines have been created to investigate Alzheimer's disease and most of the models exhibit a subset of the defining pathological features of the disease. (cellsignal.com)
  • Castellani RJ, Smith MA, Richey PL, Kalaria R, Gambetti P, Perry G (1995) Evidence for oxidative stress in Pick disease and corticobasal degeneration. (springer.com)
  • Christen Y (2000) Oxidative stress and Alzheimer's disease. (springer.com)
  • Once symptoms occur, typically in the patient's mid-40s, the disease is rapidly progressive and fatal. (redorbit.com)
  • Many people with a type of cancer called histiocytosis , which arises from mutated macrophages in tissues, eventually develop neurodegenerative symptoms many years later. (mskcc.org)
  • In fruit flies that were genetically modified to develop Alzheimer's disease symptoms, the administration of seaweed extract prolonged median life span by two days and prickly pear extract was associated with a four day extension. (prohealth.com)