Hair Cells, Auditory, Outer: Sensory cells of organ of Corti. In mammals, they are usually arranged in three or four rows, and away from the core of spongy bone (the modiolus), lateral to the INNER AUDITORY HAIR CELLS and other supporting structures. Their cell bodies and STEREOCILIA increase in length from the cochlear base toward the apex and laterally across the rows, allowing differential responses to various frequencies of sound.Motor Neurons: Neurons which activate MUSCLE CELLS.Motor Neuron Disease: 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)Neurons: 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.Survival of Motor Neuron 1 Protein: A SMN complex protein that is essential for the function of the SMN protein complex. In humans the protein is encoded by a single gene found near the inversion telomere of a large inverted region of CHROMOSOME 5. Mutations in the gene coding for survival of motor neuron 1 protein may result in SPINAL MUSCULAR ATROPHIES OF CHILDHOOD.Neurons, Afferent: Neurons which conduct NERVE IMPULSES to the CENTRAL NERVOUS SYSTEM.Motor Cortex: Area of the FRONTAL LOBE concerned with primary motor control located in the dorsal PRECENTRAL GYRUS immediately anterior to the central sulcus. It is comprised of three areas: the primary motor cortex located on the anterior paracentral lobule on the medial surface of the brain; the premotor cortex located anterior to the primary motor cortex; and the supplementary motor area located on the midline surface of the hemisphere anterior to the primary motor cortex.Survival of Motor Neuron 2 Protein: A SMN complex protein that is closely-related to SURVIVAL OF MOTOR NEURON 1 PROTEIN. In humans, the protein is encoded by an often duplicated gene found near the inversion centromere of a large inverted region of CHROMOSOME 5.Evoked Potentials, Motor: The electrical response evoked in a muscle or motor nerve by electrical or magnetic stimulation. Common methods of stimulation are by transcranial electrical and TRANSCRANIAL MAGNETIC STIMULATION. It is often used for monitoring during neurosurgery.Amyotrophic Lateral Sclerosis: 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)Spinal Cord: A cylindrical column of tissue that lies within the vertebral canal. It is composed of WHITE MATTER and GRAY MATTER.Muscular Atrophy, Spinal: 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)Motor Activity: The physical activity of a human or an animal as a behavioral phenomenon.Axons: Nerve fibers that are capable of rapidly conducting impulses away from the neuron cell body.SMN Complex Proteins: A complex of proteins that assemble the SNRNP CORE PROTEINS into a core structure that surrounds a highly conserved RNA sequence found in SMALL NUCLEAR RNA. They are found localized in the GEMINI OF COILED BODIES and in the CYTOPLASM. The SMN complex is named after the Survival of Motor Neuron Complex Protein 1, which is a critical component of the complex.Action Potentials: Abrupt changes in the membrane potential that sweep along the CELL MEMBRANE of excitable cells in response to excitation stimuli.Nerve Tissue ProteinsMolecular Motor Proteins: Proteins that are involved in or cause CELL MOVEMENT such as the rotary structures (flagellar motor) or the structures whose movement is directed along cytoskeletal filaments (MYOSIN; KINESIN; and DYNEIN motor families).Electric Stimulation: Use of electric potential or currents to elicit biological responses.Nerve Degeneration: 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.Synapses: 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.Neuromuscular Diseases: A general term encompassing lower MOTOR NEURON DISEASE; PERIPHERAL NERVOUS SYSTEM DISEASES; and certain MUSCULAR DISEASES. Manifestations include MUSCLE WEAKNESS; FASCICULATION; muscle ATROPHY; SPASM; MYOKYMIA; MUSCLE HYPERTONIA, myalgias, and MUSCLE HYPOTONIA.Ganglia, Invertebrate: Clusters of neuronal cell bodies in invertebrates. Invertebrate ganglia may also contain neuronal processes and non-neuronal supporting cells. Many invertebrate ganglia are favorable subjects for research because they have small numbers of functional neuronal types which can be identified from one animal to another.Electrophysiology: The study of the generation and behavior of electrical charges in living organisms particularly the nervous system and the effects of electricity on living organisms.Interneurons: Most generally any NEURONS which are not motor or sensory. Interneurons may also refer to neurons whose AXONS remain within a particular brain region in contrast to projection neurons, which have axons projecting to other brain regions.Synaptic Transmission: The communication from a NEURON to a target (neuron, muscle, or secretory cell) across a SYNAPSE. In chemical synaptic transmission, the presynaptic neuron releases a NEUROTRANSMITTER that diffuses across the synaptic cleft and binds to specific synaptic receptors, activating them. The activated receptors modulate specific ion channels and/or second-messenger systems in the postsynaptic cell. In electrical synaptic transmission, electrical signals are communicated as an ionic current flow across ELECTRICAL SYNAPSES.Movement: The act, process, or result of passing from one place or position to another. It differs from LOCOMOTION in that locomotion is restricted to the passing of the whole body from one place to another, while movement encompasses both locomotion but also a change of the position of the whole body or any of its parts. Movement may be used with reference to humans, vertebrate and invertebrate animals, and microorganisms. Differentiate also from MOTOR ACTIVITY, movement associated with behavior.Cholinergic Neurons: Neurons whose primary neurotransmitter is ACETYLCHOLINE.Cells, Cultured: 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.Aplysia: An opisthobranch mollusk of the order Anaspidea. It is used frequently in studies of nervous system development because of its large identifiable neurons. Aplysiatoxin and its derivatives are not biosynthesized by Aplysia, but acquired by ingestion of Lyngbya (seaweed) species.Mice, Transgenic: Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.Dopaminergic Neurons: Neurons whose primary neurotransmitter is DOPAMINE.Superoxide Dismutase: 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 126.96.36.199.Electromyography: Recording of the changes in electric potential of muscle by means of surface or needle electrodes.Anterior Horn Cells: MOTOR NEURONS in the anterior (ventral) horn of the SPINAL CORD which project to SKELETAL MUSCLES.Neuromuscular Junction: The synapse between a neuron and a muscle.Dendrites: Extensions of the nerve cell body. They are short and branched and receive stimuli from other NEURONS.Sensory Receptor Cells: Specialized afferent neurons capable of transducing sensory stimuli into NERVE IMPULSES to be transmitted to the CENTRAL NERVOUS SYSTEM. Sometimes sensory receptors for external stimuli are called exteroceptors; for internal stimuli are called interoceptors and proprioceptors.Bulbar Palsy, Progressive: A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. The adult form of the disease is marked initially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Eventually this condition may become indistinguishable from AMYOTROPHIC LATERAL SCLEROSIS. Fazio-Londe syndrome is an inherited form of this illness which occurs in children and young adults. (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900)Ganglia, Spinal: Sensory ganglia located on the dorsal spinal roots within the vertebral column. The spinal ganglion cells are pseudounipolar. The single primary branch bifurcates sending a peripheral process to carry sensory information from the periphery and a central branch which relays that information to the spinal cord or brain.Neural Inhibition: The function of opposing or restraining the excitation of neurons or their target excitable cells.GABAergic Neurons: Neurons whose primary neurotransmitter is GAMMA-AMINOBUTYRIC ACID.Patch-Clamp Techniques: An electrophysiologic technique for studying cells, cell membranes, and occasionally isolated organelles. All patch-clamp methods rely on a very high-resistance seal between a micropipette and a membrane; the seal is usually attained by gentle suction. The four most common variants include on-cell patch, inside-out patch, outside-out patch, and whole-cell clamp. Patch-clamp methods are commonly used to voltage clamp, that is control the voltage across the membrane and measure current flow, but current-clamp methods, in which the current is controlled and the voltage is measured, are also used.Immunohistochemistry: Histochemical localization of immunoreactive substances using labeled antibodies as reagents.Models, Neurological: Theoretical representations that simulate the behavior or activity of the neurological system, processes or phenomena; includes the use of mathematical equations, computers, and other electronic equipment.Brain: 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.Cerebral Cortex: 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.Animals, Newborn: Refers to animals in the period of time just after birth.Disease Models, Animal: Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Facial Nerve: The 7th cranial nerve. The facial nerve has two parts, the larger motor root which may be called the facial nerve proper, and the smaller intermediate or sensory root. Together they provide efferent innervation to the muscles of facial expression and to the lacrimal and SALIVARY GLANDS, and convey afferent information for TASTE from the anterior two-thirds of the TONGUE and for TOUCH from the EXTERNAL EAR.Membrane Potentials: The voltage differences across a membrane. For cellular membranes they are computed by subtracting the voltage measured outside the membrane from the voltage measured inside the membrane. They result from differences of inside versus outside concentration of potassium, sodium, chloride, and other ions across cells' or ORGANELLES membranes. For excitable cells, the resting membrane potentials range between -30 and -100 millivolts. Physical, chemical, or electrical stimuli can make a membrane potential more negative (hyperpolarization), or less negative (depolarization).Motor Skills Disorders: Marked impairments in the development of motor coordination such that the impairment interferes with activities of daily living. (From DSM-V)Animals, Genetically Modified: ANIMALS whose GENOME has been altered by GENETIC ENGINEERING, or their offspring.Hippocampus: 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.gamma-Aminobutyric Acid: The most common inhibitory neurotransmitter in the central nervous system.Brain Stem: The part of the brain that connects the CEREBRAL HEMISPHERES with the SPINAL CORD. It consists of the MESENCEPHALON; PONS; and MEDULLA OBLONGATA.Axonal Transport: 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)Neuropeptides: Peptides released by NEURONS as intercellular messengers. Many neuropeptides are also hormones released by non-neuronal cells.Mutation: 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.Neural Conduction: The propagation of the NERVE IMPULSE along the nerve away from the site of an excitation stimulus.Choline O-Acetyltransferase: An enzyme that catalyzes the formation of acetylcholine from acetyl-CoA and choline. EC 188.8.131.52.Ganglia: Clusters of multipolar neurons surrounded by a capsule of loosely organized CONNECTIVE TISSUE located outside the CENTRAL NERVOUS SYSTEM.Grasshoppers: Plant-eating orthopterans having hindlegs adapted for jumping. There are two main families: Acrididae and Romaleidae. Some of the more common genera are: Melanoplus, the most common grasshopper; Conocephalus, the eastern meadow grasshopper; and Pterophylla, the true katydid.Neurons, Efferent: Neurons which send impulses peripherally to activate muscles or secretory cells.Nerve Net: A meshlike structure composed of interconnecting nerve cells that are separated at the synaptic junction or joined to one another by cytoplasmic processes. In invertebrates, for example, the nerve net allows nerve impulses to spread over a wide area of the net because synapses can pass information in any direction.Neurofilament Proteins: 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)Locomotion: Movement or the ability to move from one place or another. It can refer to humans, vertebrate or invertebrate animals, and microorganisms.Central Nervous System: The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges.Reaction Time: The time from the onset of a stimulus until a response is observed.Green Fluorescent Proteins: 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.Behavior, Animal: The observable response an animal makes to any situation.Medulla Oblongata: The lower portion of the BRAIN STEM. It is inferior to the PONS and anterior to the CEREBELLUM. Medulla oblongata serves as a relay station between the brain and the spinal cord, and contains centers for regulating respiratory, vasomotor, cardiac, and reflex activities.Excitatory Postsynaptic Potentials: Depolarization of membrane potentials at the SYNAPTIC MEMBRANES of target neurons during neurotransmission. Excitatory postsynaptic potentials can singly or in summation reach the trigger threshold for ACTION POTENTIALS.Neuronal Plasticity: The capacity of the NERVOUS SYSTEM to change its reactivity as the result of successive activations.Evoked Potentials: Electrical responses recorded from nerve, muscle, SENSORY RECEPTOR, or area of the CENTRAL NERVOUS SYSTEM following stimulation. They range from less than a microvolt to several microvolts. The evoked potential can be auditory (EVOKED POTENTIALS, AUDITORY), somatosensory (EVOKED POTENTIALS, SOMATOSENSORY), visual (EVOKED POTENTIALS, VISUAL), or motor (EVOKED POTENTIALS, MOTOR), or other modalities that have been reported.Cell Count: The number of CELLS of a specific kind, usually measured per unit volume or area of sample.Mice, Inbred C57BLMuscle, Skeletal: A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.Gene Expression Regulation, Developmental: Any of the processes by which nuclear, cytoplasmic, or intercellular factors influence the differential control of gene action during the developmental stages of an organism.Olfactory Receptor Neurons: Neurons in the OLFACTORY EPITHELIUM with proteins (RECEPTORS, ODORANT) that bind, and thus detect, odorants. These neurons send their DENDRITES to the surface of the epithelium with the odorant receptors residing in the apical non-motile cilia. Their unmyelinated AXONS synapse in the OLFACTORY BULB of the BRAIN.Glutamic Acid: A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.Psychomotor Performance: The coordination of a sensory or ideational (cognitive) process and a motor activity.Cats: The domestic cat, Felis catus, of the carnivore family FELIDAE, comprising over 30 different breeds. The domestic cat is descended primarily from the wild cat of Africa and extreme southwestern Asia. Though probably present in towns in Palestine as long ago as 7000 years, actual domestication occurred in Egypt about 4000 years ago. (From Walker's Mammals of the World, 6th ed, p801)Afferent Pathways: Nerve structures through which impulses are conducted from a peripheral part toward a nerve center.Dopamine: 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.Leeches: Annelids of the class Hirudinea. Some species, the bloodsuckers, may become temporarily parasitic upon animals, including man. Medicinal leeches (HIRUDO MEDICINALIS) have been used therapeutically for drawing blood since ancient times.Cyclic AMP Response Element-Binding Protein: A protein that has been shown to function as a calcium-regulated transcription factor as well as a substrate for depolarization-activated CALCIUM-CALMODULIN-DEPENDENT PROTEIN KINASES. This protein functions to integrate both calcium and cAMP signals.Chick Embryo: The developmental entity of a fertilized chicken egg (ZYGOTE). The developmental process begins about 24 h before the egg is laid at the BLASTODISC, a small whitish spot on the surface of the EGG YOLK. After 21 days of incubation, the embryo is fully developed before hatching.Rats, Sprague-Dawley: 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.Muscles: Contractile tissue that produces movement in animals.Neuroglia: 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.Rhombencephalon: The posterior of the three primitive cerebral vesicles of an embryonic brain. It consists of myelencephalon, metencephalon, and isthmus rhombencephali from which develop the major BRAIN STEM components, such as MEDULLA OBLONGATA from the myelencephalon, CEREBELLUM and PONS from the metencephalon, with the expanded cavity forming the FOURTH VENTRICLE.Paralysis: A general term most often used to describe severe or complete loss of muscle strength due to motor system disease from the level of the cerebral cortex to the muscle fiber. This term may also occasionally refer to a loss of sensory function. (From Adams et al., Principles of Neurology, 6th ed, p45)Analysis of Variance: A statistical technique that isolates and assesses the contributions of categorical independent variables to variation in the mean of a continuous dependent variable.Pyramidal Cells: Projection neurons in the CEREBRAL CORTEX and the HIPPOCAMPUS. Pyramidal cells have a pyramid-shaped soma with the apex and an apical dendrite pointed toward the pial surface and other dendrites and an axon emerging from the base. The axons may have local collaterals but also project outside their cortical region.Spinal Muscular Atrophies of Childhood: A group of recessively inherited diseases that feature progressive muscular atrophy and hypotonia. They are classified as type I (Werdnig-Hoffman disease), type II (intermediate form), and type III (Kugelberg-Welander disease). Type I is fatal in infancy, type II has a late infantile onset and is associated with survival into the second or third decade. Type III has its onset in childhood, and is slowly progressive. (J Med Genet 1996 Apr:33(4):281-3)Rats, Wistar: A strain of albino rat developed at the Wistar Institute that has spread widely at other institutions. This has markedly diluted the original strain.Cell Differentiation: Progressive restriction of the developmental potential and increasing specialization of function that leads to the formation of specialized cells, tissues, and organs.Cerebellum: 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.Astrocytes: 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.Neurogenesis: Formation of NEURONS which involves the differentiation and division of STEM CELLS in which one or both of the daughter cells become neurons.Periodicity: The tendency of a phenomenon to recur at regular intervals; in biological systems, the recurrence of certain activities (including hormonal, cellular, neural) may be annual, seasonal, monthly, daily, or more frequently (ultradian).Nerve Growth Factors: Factors which enhance the growth potentialities of sensory and sympathetic nerve cells.Mice, Knockout: 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.Physical Stimulation: Act of eliciting a response from a person or organism through physical contact.Mesencephalon: 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.Peripheral Nerves: The nerves outside of the brain and spinal cord, including the autonomic, cranial, and spinal nerves. Peripheral nerves contain non-neuronal cells and connective tissue as well as axons. The connective tissue layers include, from the outside to the inside, the epineurium, the perineurium, and the endoneurium.FMRFamide: A molluscan neuroactive peptide which induces a fast excitatory depolarizing response due to direct activation of amiloride-sensitive SODIUM CHANNELS. (From Nature 1995; 378(6558): 730-3)Serotonin: A biochemical messenger and regulator, synthesized from the essential amino acid L-TRYPTOPHAN. In humans it is found primarily in the central nervous system, gastrointestinal tract, and blood platelets. Serotonin mediates several important physiological functions including neurotransmission, gastrointestinal motility, hemostasis, and cardiovascular integrity. Multiple receptor families (RECEPTORS, SEROTONIN) explain the broad physiological actions and distribution of this biochemical mediator.Nerve Regeneration: Renewal or physiological repair of damaged nerve tissue.Neurites: 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.Cell Death: The termination of the cell's ability to carry out vital functions such as metabolism, growth, reproduction, responsiveness, and adaptability.Kinesin: A microtubule-associated mechanical adenosine triphosphatase, that uses the energy of ATP hydrolysis to move organelles along microtubules toward the plus end of the microtubule. The protein is found in squid axoplasm, optic lobes, and in bovine brain. Bovine kinesin is a heterotetramer composed of two heavy (120 kDa) and two light (62 kDa) chains. EC 3.6.1.-.Axotomy: Transection or severing of an axon. This type of denervation is used often in experimental studies on neuronal physiology and neuronal death or survival, toward an understanding of nervous system disease.Cell Survival: 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.Signal Transduction: 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.Functional Laterality: Behavioral manifestations of cerebral dominance in which there is preferential use and superior functioning of either the left or the right side, as in the preferred use of the right hand or right foot.Recruitment, Neurophysiological: The spread of response if stimulation is prolonged. (Campbell's Psychiatric Dictionary, 8th ed.)Nervous System: 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)Tetrodotoxin: An aminoperhydroquinazoline poison found mainly in the liver and ovaries of fishes in the order TETRAODONTIFORMES, which are eaten. The toxin causes paresthesia and paralysis through interference with neuromuscular conduction.Cranial Nerves: Twelve pairs of nerves that carry general afferent, visceral afferent, special afferent, somatic efferent, and autonomic efferent fibers.Caenorhabditis elegans: A species of nematode that is widely used in biological, biochemical, and genetic studies.Homeodomain Proteins: Proteins encoded by homeobox genes (GENES, HOMEOBOX) that exhibit structural similarity to certain prokaryotic and eukaryotic DNA-binding proteins. Homeodomain proteins are involved in the control of gene expression during morphogenesis and development (GENE EXPRESSION REGULATION, DEVELOPMENTAL).In Situ Hybridization: A technique that localizes specific nucleic acid sequences within intact chromosomes, eukaryotic cells, or bacterial cells through the use of specific nucleic acid-labeled probes.Presynaptic Terminals: The distal terminations of axons which are specialized for the release of neurotransmitters. Also included are varicosities along the course of axons which have similar specializations and also release transmitters. Presynaptic terminals in both the central and peripheral nervous systems are included.Sciatic Nerve: A nerve which originates in the lumbar and sacral spinal cord (L4 to S3) and supplies motor and sensory innervation to the lower extremity. The sciatic nerve, which is the main continuation of the sacral plexus, is the largest nerve in the body. It has two major branches, the TIBIAL NERVE and the PERONEAL NERVE.Zebrafish: An exotic species of the family CYPRINIDAE, originally from Asia, that has been introduced in North America. They are used in embryological studies and to study the effects of certain chemicals on development.RNA-Binding Proteins: Proteins that bind to RNA molecules. Included here are RIBONUCLEOPROTEINS and other proteins whose function is to bind specifically to RNA.Serotonergic Neurons: Neurons whose primary neurotransmitter is SEROTONIN.Astacoidea: A superfamily of various freshwater CRUSTACEA, in the infraorder Astacidea, comprising the crayfish. Common genera include Astacus and Procambarus. Crayfish resemble lobsters, but are usually much smaller.Muscle Contraction: A process leading to shortening and/or development of tension in muscle tissue. Muscle contraction occurs by a sliding filament mechanism whereby actin filaments slide inward among the myosin filaments.Calcium: A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.Tyrosine 3-Monooxygenase: An enzyme that catalyzes the conversion of L-tyrosine, tetrahydrobiopterin, and oxygen to 3,4-dihydroxy-L-phenylalanine, dihydrobiopterin, and water. EC 184.108.40.206.Efferent Pathways: Nerve structures through which impulses are conducted from a nerve center toward a peripheral site. Such impulses are conducted via efferent neurons (NEURONS, EFFERENT), such as MOTOR NEURONS, autonomic neurons, and hypophyseal neurons.Extremities: The farthest or outermost projections of the body, such as the HAND and FOOT.Reflex: An involuntary movement or exercise of function in a part, excited in response to a stimulus applied to the periphery and transmitted to the brain or spinal cord.Neural Pathways: Neural tracts connecting one part of the nervous system with another.Macaca mulatta: A species of the genus MACACA inhabiting India, China, and other parts of Asia. The species is used extensively in biomedical research and adapts very well to living with humans.Neurotransmitter Agents: Substances used for their pharmacological actions on any aspect of neurotransmitter systems. Neurotransmitter agents include agonists, antagonists, degradation inhibitors, uptake inhibitors, depleters, precursors, and modulators of receptor function.LIM-Homeodomain Proteins: A subclass of LIM domain proteins that include an additional centrally-located homeodomain region that binds AT-rich sites on DNA. Many LIM-homeodomain proteins play a role as transcriptional regulators that direct cell fate.Caenorhabditis elegans Proteins: Proteins from the nematode species CAENORHABDITIS ELEGANS. The proteins from this species are the subject of scientific interest in the area of multicellular organism MORPHOGENESIS.Embryo, Mammalian: The entity of a developing mammal (MAMMALS), generally from the cleavage of a ZYGOTE to the end of embryonic differentiation of basic structures. For the human embryo, this represents the first two months of intrauterine development preceding the stages of the FETUS.Molecular Sequence Data: 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.Myenteric Plexus: One of two ganglionated neural networks which together form the ENTERIC NERVOUS SYSTEM. The myenteric (Auerbach's) plexus is located between the longitudinal and circular muscle layers of the gut. Its neurons project to the circular muscle, to other myenteric ganglia, to submucosal ganglia, or directly to the epithelium, and play an important role in regulating and patterning gut motility. (From FASEB J 1989;3:127-38)Movement Disorders: Syndromes which feature DYSKINESIAS as a cardinal manifestation of the disease process. Included in this category are degenerative, hereditary, post-infectious, medication-induced, post-inflammatory, and post-traumatic conditions.Brain Mapping: Imaging techniques used to colocalize sites of brain functions or physiological activity with brain structures.Thalamus: Paired bodies containing mostly GRAY MATTER and forming part of the lateral wall of the THIRD VENTRICLE of the brain.Hand: The distal part of the arm beyond the wrist in humans and primates, that includes the palm, fingers, and thumb.Brain-Derived Neurotrophic Factor: 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)Pons: The front part of the hindbrain (RHOMBENCEPHALON) that lies between the MEDULLA and the midbrain (MESENCEPHALON) ventral to the cerebellum. It is composed of two parts, the dorsal and the ventral. The pons serves as a relay station for neural pathways between the CEREBELLUM to the CEREBRUM.Growth Cones: Bulbous enlargement of the growing tip of nerve axons and dendrites. They are crucial to neuronal development because of their pathfinding ability and their role in synaptogenesis.RNA-Binding Protein FUS: 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.Spinal Nerve Roots: Paired bundles of NERVE FIBERS entering and leaving the SPINAL CORD at each segment. The dorsal and ventral nerve roots join to form the mixed segmental spinal nerves. The dorsal roots are generally afferent, formed by the central projections of the spinal (dorsal root) ganglia sensory cells, and the ventral roots are efferent, comprising the axons of spinal motor and PREGANGLIONIC AUTONOMIC FIBERS.Substantia Nigra: 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.RNA, Messenger: 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.Proprioception: Sensory functions that transduce stimuli received by proprioceptive receptors in joints, tendons, muscles, and the INNER EAR into neural impulses to be transmitted to the CENTRAL NERVOUS SYSTEM. Proprioception provides sense of stationary positions and movements of one's body parts, and is important in maintaining KINESTHESIA and POSTURAL BALANCE.Receptors, N-Methyl-D-Aspartate: A class of ionotropic glutamate receptors characterized by affinity for N-methyl-D-aspartate. NMDA receptors have an allosteric binding site for glycine which must be occupied for the channel to open efficiently and a site within the channel itself to which magnesium ions bind in a voltage-dependent manner. The positive voltage dependence of channel conductance and the high permeability of the conducting channel to calcium ions (as well as to monovalent cations) are important in excitotoxicity and neuronal plasticity.Photic Stimulation: Investigative technique commonly used during ELECTROENCEPHALOGRAPHY in which a series of bright light flashes or visual patterns are used to elicit brain activity.Corpus Striatum: 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.
Amyotrophic lateral sclerosis research
"Cell-based therapies for amyotrophic lateral sclerosis/motor neuron disease". The Cochrane Database of Systematic Reviews. 11: ... These iPSC derived cells can be used in transplant cell therapy, in which they can introduce the differentiated cells into the ... In this strategy the disease is introduced to cell cultures in petri dishes. In this case, motor cells can be grown, and the ... the denervation of motor neurons and dysfunction of neurons can be visualized using fluorescent markers to study the ...
Amyotrophic lateral sclerosis
... "motor neurone disease", which is a group of neurological disorders that selectively affect motor neurons, the cells that ... In this case, neurons in the brain (upper motor neurons) and in the spinal cord (lower motor neurons) are dying and this form ... The mutant SOD1 may also contribute to motor neuron cell death through generating free radicals. Excitotoxicity, or cell death ... An abnormal reflex commonly called Babinski's sign also indicates upper motor neuron damage. Symptoms of lower motor neuron ...
Chromatophores are color pigment changing cells that are directly stimulated by central motor neurons. They are primarily used ... These cells are usually located beneath the skin or scale the animals. There are two categories of colors generated by the cell ... Vitiligo is a condition in which there is a loss of pigment-producing cells called melanocytes in patches of skin. Carotenoids ... To change the color pigments, transparency, or opacity, the cells alter in form and size, and stretch or contract their outer ...
Distal spinal muscular atrophy type 1
Cell therapy and stem cells in animal models of motor neuron disorders". European Journal of Neuroscience. 26 (7): 1721-1737. ... and transplantation of healthy motor neurons grown in vitro from the patient's stem cells. Studies in amyotrophic lateral ... The pathology underlying the observable characteristics of DSMA1 is cell body degeneration of motor nerves. Specifically, the ... is a rare neuromuscular disorder involving death of motor neurons in the spinal cord which leads to a generalized progressive ...
... these signals can then connect with other neurons at synapses, or to motor cells or glands. In other types of cells, their main ... muscle cells, endocrine cells, and in some plant cells. In neurons, action potentials play a central role in cell-to-cell ... Several types of cells support an action potential, such as plant cells, muscle cells, and the specialized cells of the heart ( ... the initial photoreceptor cells and the next layer of cells (comprising bipolar cells and horizontal cells) do not produce ...
Lack of Schwann cell maturation leads to degeneration of motor and sensory neurons. Excessive ErbB signaling is associated with ... This leads to decreased proliferation of tumor cells.Trastuzumad targets tumor cells and causes apoptosis through the immune ... Cell. 125 (6): 1137-1149. doi:10.1016/j.cell.2006.05.013. ISSN 0092-8674. PMID 16777603. Garrett TP, McKern NM, et al. (2002 ... Cell. 11 (2): 507-517. doi:10.1016/S1097-2765(03)00047-9. PMID 12620237. Franklin MC, Carey KD, et al. (2004). "Insights into ...
Hereditary spastic paraplegia
The affected cells are the primary motor neurons, therefore the disease is an upper motor neuron disease. HSP is not a form of ... HSP affects several pathways in motor neurons. Many genes were identified and linked to HSP. It remains a challenge to ... Dysfunction of endosomal trafficking can have severe consequences in motor neurons with long axons, as reported in HSP. ... another nerve cell or a muscle). Significant for this mechanism is the L1CAM gene, a cell surface glycoprotein of the ...
A motor unit is a nerve cell (or neuron) and the muscle fibers it activates. Poliovirus attacks specific neurons in the ... In an effort to compensate for the loss of these neurons, surviving motor neurons sprout new nerve terminals to the orphaned ... Because polio survivors have already lost a considerable number of motor neurons, further age-related loss of neurons may ... neural fatigue theory proposes that the enlargement of the motor neuron fibers places added metabolic stress on the nerve cell ...
New York Genome Center
"A Multi-step Transcriptional and Chromatin State Cascade Underlies Motor Neuron Programming from Embryonic Stem Cells". Cell ... Cell. 168 (3): 460-472.e14. doi:10.1016/j.cell.2016.12.025. PMID 28089356. "Cheryl Moore, Hilde Windels, and more". People in ... "Single-cell RNA-seq reveals new types of human blood dendritic cells, monocytes, and progenitors". Science. 356 (6335): ... Cell Systems. 4 (2): 157-170.e14. doi:10.1016/j.cels.2016.12.011. PMC 5388136 . PMID 28131822. Manier, Salomon; Salem, Karma Z ...
December 2006). "T-Box transcription factor Tbx20 regulates a genetic program for cranial motor neuron cell body migration". ... The complete elimination of Tbx20 does not cause trigeminal and facial neurons to switch to hypoglossal neurons. Facial neurons ... and a lack of transmedian migration of vestibuloacoustic cells. However, hindbrain motor neurons lacking Tbx20 retained the ... Certain neuron groups relate to Hox gene expression. At the r4 level, Hoxb1 is suspected to bestow rhombomere 4 cell identity. ...
Basal plate (neural tube)
The cell types of the basal plate include lower motor neurons and four types of interneuron. Initially, the left and right ... It extends from the rostral mesencephalon to the end of the spinal cord and contains primarily motor neurons, whereas neurons ... Differentiation of neurons in the basal plate is under the influence of the protein Sonic hedgehog released by ventralizing ...
"T-Box transcription factor Tbx20 regulates a genetic program for cranial motor neuron cell body migration". Development ( ... stromal cells shows that the expression of C8orf48 is lower in the disease state cells in comparison to healthy cells. the ... or regulation of cell migration. This implies that C8orf48 may play a role in the cell cycle. A few of the transcription ... "Protein Kinase C Signaling , Cell Signaling Technology". www.cellsignal.com. Retrieved 2016-05-09. PSICQUIC. "PSICQUIC View". ...
Juvenile primary lateral sclerosis
The disorder damages motor neurons, which are specialized nerve cells in the brain and spinal cord that control muscle movement ... It is currently unknown how the loss of functional alsin protein causes the death of motor neurons and the symptoms of juvenile ... Alsin is abundant in motor neurons, but its function is not fully understood. Mutations in the ALS2 gene in this disorder ... which means two copies of the gene in each cell are altered. Most often, parents of affected individuals each carry one copy of ...
... of novel spinal cholinergic genetic subtypes disclose Chodl and Pitx2 as markers for fast motor neurons and partition cells". J ... The exact function of chondrolectin is unknown but it has been show to be a marker of fast motor neurons in mice, and is ... human chondrolectin has been shown to localise to motor neurons within the spinal cord. Chondrolectin is alternatively spliced ... which predominantly affects lower motor neurons. Increased levels of chondrolectin in a zebrafish model of SMA results in ...
... the motor neuron, the myofiber, and the Schwann cell. In a normally functioning synapse, a signal will cause the motor neuron ... Wnt-3 is expressed by muscle fibers and is secreted retrogradely onto motor neurons. In motor neurons, Wnt-3 works with Agrin ... This brain region contains three main neuronal cell types- Purkinje cells, granule cells and mossy fiber cells. Wnt-3 ... In the vertebrate NMJ, motor neuron expression of Wnt-11r contributes to acetylcholine receptor (AChR) clustering in the ...
Anterior grey column
The anterior grey column is the column where the cell bodies of alpha motor neurons are located. The anterior grey column, ... of alpha motor neurons via interaction with gamma fibers. Alpha motor neuron Beta motor neuron Gamma motor neuron Section of ... The anterior grey column contains motor neurons that affect the skeletal muscles while the posterior grey column receives ... It is these cells that are affected in the following diseases, - amyotrophic lateral sclerosis, Charcot-Marie- ...
"Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model". Nature Neuroscience. 10 (5): ... but the findings are significant because they implicate cells other than neuron cells in neurodegeneration. Batten disease is a ... Astrocytes then cause the toxic effects on the motor neurons. The specific mechanism of toxicity still needs to be investigated ... Amyotrophic lateral sclerosis (ALS or Lou Gehrig's disease) is a disease in which motor neurons are selectively targeted for ...
Manoj Kumar Jaiswal
... of induced pluripotent stem cells-derived motor neurons for treatment of amyotrophic lateral sclerosis and motor neuron disease ... recent advances gained from genetically modified animals and cell culture models. In Motor Neuron Diseases: Causes, ... "Riluzole But Not Melatonin Ameliorates Acute Motor Neuron Degeneration and Moderately Inhibits SOD1-Mediated Excitotoxicity ... His lab work is conducted on mice, induced pluripotent stem cells (iPSC) and Human postmortem tissue. Dr. Jaiswal's recent ...
Cell. 80 (1): 155-65. doi:10.1016/0092-8674(95)90460-3. PMID 7813012. "Entrez Gene: SMN1 survival of motor neuron 1, telomeric ... Survival of motor neuron 1 (SMN1), also known as component of gems 1 or GEMIN1, is a gene that encodes the SMN protein in ... Liu Q, Dreyfuss G (July 1996). "A novel nuclear structure containing the survival of motor neurons protein". The EMBO Journal. ... "The survival motor neuron protein in spinal muscular atrophy". Human Molecular Genetics. 6 (8): 1205-14. doi:10.1093/hmg/6.8. ...
After fragmentation, degradation of the cell begins, leading to a loss of both motor neurons and dorsal root ganglia. Symptoms ... Any disorder that compromises the synaptic transmission between a motor neuron and a muscle cell is categorized under the ... The development of the neuromuscular junction requires signaling from both the motor neuron's terminal and the muscle cell's ... ACh release by developing motor neurons produces postsynaptic potentials in the muscle cell that positively reinforces the ...
"Nkx2.2 and Nkx2.9 Are the Key Regulators to Determine Cell Fate of Branchial and Visceral Motor Neurons in Caudal Hindbrain". ... Nkx 2.9 works together with another transcription factor, Nkx 2.2, to direct neural progenitor cells to their cell fate. Cell ... Nkx 2.9 is a transcription factor responsible for the formation of the branchial and visceral motor neuron subtypes of cranial ... that Nkx 2.9 and Nkx 2.2 proteins do not play a role in branchial or visceral motor neuron development in the portion of the ...
... dorsal differentiation of pre-neural cells into sensory neurons and Shh promotes ventral differentiation into motor neurons. ... Neuron. 68 (2): 245-53. doi:10.1016/j.neuron.2010.09.042. PMC 3010396 . PMID 20955932. "This Week In the Journal." The Journal ... Due to the graded response the cells of the neural tube have to BMP and Shh signaling, these pathways are in competition to ... Inhibitors of BMPs, such as NOG and CHRD, promote differentiation of ectoderm cells into prospective neural tissue on the ...
... and normal numbers of cranial neural crest cells, motor neurons and axons. NEDD4 has been shown to interact with and ... on the cell surface. The deletion of NEDD4 in mice leads to a reduced number of effector T-cells, and a slower T-cell response ... Neuron. 65 (3): 341-57. doi:10.1016/j.neuron.2010.01.017. PMC 2862300 . PMID 20159448. Hong SW, Moon JH, Kim JS, Shin JS, Jung ... Cell. 128 (1): 141-56. doi:10.1016/j.cell.2006.11.040. PMC 1855245 . PMID 17218261. Fouladkou F, Landry T, Kawabe H, Neeb A, Lu ...
The movement problems are caused by impairment of motor neurons, which are specialized nerve cells in the brain and spinal cord ... Collins FS, Rossant J, Wurst W (Jan 2007). "A mouse for all reasons". Cell. 128 (1): 9-13. doi:10.1016/j.cell.2006.12.018. PMID ... When the red cells are broken down, iron and a molecule called bilirubin are released; individuals with triosephosphate ... These immune system cells normally recognize and attack foreign invaders, such as viruses and bacteria, to prevent infection. ...
West Nile fever
Disconnect of upper motor neuron influences on the anterior horn cells possibly by myelitis or glutamate excitotoxicity have ... A prominent finding in WNE is muscular weakness (30 to 50 percent of patients with encephalitis), often with lower motor neuron ... Pleocytosis, an increase of white blood cells in cerebrospinal fluid, is also present. Changes in consciousness are not usually ... Morpholino antisense oligos conjugated to cell penetrating peptides have been shown to partially protect mice from WNV disease ...
Don W. Cleveland
Cleveland's research looks at the molecular genetics of axonal growth and motor neuron disease and the cell biology of ... "President's Column" (PDF). The American Society for Cell Biology. The American Society for Cell Biology. "The Sheila Essey ... Cell and Molecular Biology of the Cytoskeleton: Molecular Mechanisms Controlling Tubulin Synthesis Edited by Jerry W. Shay ( ... Cleveland has made pioneering discoveries of the mechanisms of chromosome movement and cell-cycle control during normal ...
Rats that have had the motor neurons in the brain stem disconnected from the neural circuits of the cerebral hemispheres ( ... The brain detects insulin in the blood, which indicates that nutrients are being absorbed by cells and a person is getting full ... When the glucose levels of cells drop (glucoprivation), the body starts to produce the feeling of hunger. The body also ...
The TH2 lymphocytes interact with B cells and together they produce IgE. IgE circulates around and binds to receptors of cells ... In "kindling", repeated stimulation of hippocampal or amygdaloid neurons in the limbic system eventually leads to seizures in ... such as sensitization to the locomotor response of a stimulant resulting in cross-sensitization to the motor-activating effects ... In "central sensitization," nociceptive neurons in the dorsal horns of the spinal cord become sensitized by peripheral tissue ...
This is due to the reduction of excitatory synaptic transmission in a nucleus and increased excitability in motor neurons ... It also contains pacemaker cells and nonpacemaker cells that initiate spontaneous breathing. Research is being conducted on the ... There are two types of neurons in the pre-BötC: nonpacemaker and pacemaker neurons. Nonpacemaker neurons enter either a tonic ... intrinsically-bursting pacemaker neurons, and follower neurons within the pre-Bötzinger complex. Together these neurons make up ...
The skin consists of a thin outer epidermis with mucous cells and sensory cells, and a connective tissue dermis consisting ... Zullo, L.; Sumbre, G.; Agnisola, C.; Flash, T.; Hochner, B. (2009). "Nonsomatotopic organization of the higher motor centers in ... Two-thirds of an octopus's neurons are found in the nerve cords of its arms, which show a variety of complex reflex actions ... Other colour-changing cells are reflective iridophores and white leucophores. This colour-changing ability is also used to ...
The motor part of the spindle is provided by motor neurons: up to a dozen gamma motor neurons and one or two beta motor neurons ... Gamma motor neurons supply only muscle fibres within the spindle, whereas beta motor neurons supply muscle fibres both within ... muscle fibres within the spindle by up to a dozen gamma motor neurons and to a lesser extent by one or two beta motor neurons[ ... The function of the gamma motor neurons is not to supplement the force of muscle contraction provided by the extrafusal fibers ...
Theodore Holmes Bullock
In one series of famous experiments on the cardiac ganglion in lobsters, Bullock demonstrated that neurons can communicate not ... This idea, that electrical synapses couple groups of cells into functional units, lead to Bullock's lifelong interest in field ... is an excellent example of how motor programs are integrated with incoming sensory information when generating a behavior ... potentials, which are generated by the summated electrical activity of millions of brain cells. Bullock was a respected teacher ...
Neuroscience of music
Mirror/echo neurons and auditory-motor interactions. The mirror neuron system has an important role in neural models of ... The hair cells in the cochlea release neurotransmitter as a result, causing action potentials down the auditory nerve. The ... The motor area processes the rhythm of the music (Dean, 2013). The motor area of the brain is located in the ... Auditory-motor interactions. Feedforward and feedback interactions. An auditory-motor interaction may be loosely ...
Cell Physiology. 281 (5): C1596-603. doi:10.1152/ajpcell.2001.281.5.c1596. PMID 11600423.. ... If oxygen deprivation continues, cognitive disturbances, and decreased motor control will result. The skin may also appear ... this may be due to an autoimmune response caused by carbon monoxide-induced changes in the myelin sheath surrounding neurons.[ ... Brain cells are very sensitive to reduced oxygen levels. Once deprived of oxygen they will begin to die off within five minutes ...
Some of the branches of the I-a axons synapse directly with alpha motor neurons.These carry impulses back to the same muscle ... Indeed, the most sensitive mechanoreceptors in humans are the hair cells in the cochlea of the inner ear (no relation to the ... These, in turn, synapse with motor neurons leading back to the antagonistic muscle, a flexor in the back of the thigh. By ... where second-order neurons send the signal to the thalamus and synapse with third-order neurons in the ventrobasal complex. The ...
Brain-derived neurotrophic factor
negative regulation of neuron apoptotic process. • synapse assembly. • cell-cell signaling. • positive regulation of brain- ... motor neurons and skeletal muscle and it is also found in saliva. ... regulation of neuron differentiation. • neuron projection morphogenesis. • modulation of chemical synaptic transmission. • ... parts of the adult brain retain the ability to grow new neurons from neural stem cells in a process known as neurogenesis. ...
Mutations in the first SOD enzyme (SOD1) can cause familial amyotrophic lateral sclerosis (ALS, a form of motor neuron disease ... "The expression of different superoxide dismutase forms is cell-type dependent in olive (Olea europaea L.) leaves". Plant & Cell ... The cytosols of virtually all eukaryotic cells contain an SOD enzyme with copper and zinc (Cu-Zn-SOD). For example, Cu-Zn-SOD ... Human white blood cells use enzymes such as NADPH oxidase to generate superoxide and other reactive oxygen species to kill ...
নিতম্বাস্থি - উইকিপিডিয়া
বহির্বাহী স্নায়ু (Efferent nerve) / চেষ্টীয় স্নায়ু (Motor nerve). *মিশ্র স্নায়ু (Mixed nerve) ... আলোকগ্রাহক কোষ (Photoreceptor cell). *দণ্ড কোষ (Rod cell). *শঙ্কু কোষ (Cone cell) ... স্নায়ুকোষ (Neuron). *স্নায়ু অক্ষ (Axon). *স্নায়ুপ্রশাখা (Dendrite). *স্নায়ুসন্নিধি (Synapse). *স্নায়ুধারীয় বর্জ্য ...
Olfactory ensheathing cells
... myelinated axons of olfactory neurons in a similar way to which Schwann cells ensheath non-myelinated peripheral neurons. They ... Traumatic spinal cord damage causes a permanent loss of motor and sensory functions in the central nervous system, termed ... be rejected by the body and biological functions such as cell adhesion and growth will be enhanced through cell-cell and cell- ... "CD46 on glial cells can function as a receptor for viral glycoprotein-mediated cell-cell fusion". Glia. 52 (3): 252-8. doi: ...
Outline of brain mapping
Neurons are generated by cell division.. *Neurons are connected by sites of contact and not via cytoplasmic continuity. (A cell ... cognitive or motor) event stimuli. The voltage swing sequences are recorded and broken down by positive and negative, and by ... The Neuron doctrine postulates several elementary aspects of neurons: *The brain is made up of individual cells (neurons) that ... Every neuron has a nucleus, which is the trophic center of the cell (The part which must have access to nutrition). If the cell ...
Amyotrophic lateral sclerosis
... "motor neurone disease", which is a group of neurological disorders that selectively affect motor neurons, the cells that ... Typical or "classical" ALS involves neurons in the brain (upper motor neurons) and in the spinal cord (lower motor neurons).[27 ... Typical or "classical" ALS involves neurons in the brain (upper motor neurons) and in the spinal cord (lower motor neurons).[27 ... The defining feature of ALS is the death of both upper motor neurons (located in the motor cortex of the brain) and lower motor ...
... some cell lines - in particular, primary cultures of quiescent and differentiated cells such as thymocytes and neurons - are ... Ikeda K, Akiyama H, Arai T, Ueno H, Tsuchiya K, Kosaka K (July 2002). "Morphometrical reappraisal of motor neuron system of ... "Cell. 137 (1): 133-45. doi:10.1016/j.cell.2009.01.041. PMC 2668214. PMID 19345192.. ... Cell cycle controlEdit. Cell cycle progression is controlled by ordered action of cyclin-dependent kinases (CDKs), activated by ...
High concentrations are reached in neocortical, limbic, sensory and motor areas.. *^ Huestis MA (August 2007). "Human ... mainly expressed in cells of the immune system. The psychoactive effects of THC are primarily mediated by the activation of ... possesses mild antioxidant activity sufficient to protect neurons against oxidative stress, such as that produced by glutamate- ...
... in affected neurons that cells cannot break down. Deregulation of the autophagy pathway and mutation of alleles regulating ... The Dynamic Interaction of Ambra1 with the Dynein Motor Complex Regulates Mammalian Autophagy', J Cell Biol, 191 (2010), 155-68 ... Mechanism of cell death. Cells that undergo an extreme amount of stress experience cell death either through apoptosis or ... "Cannabisin B induces autophagic cell death by inhibiting the AKT/mTOR pathway and S phase cell cycle arrest in HepG2 cells". ...
Neurons are cells specialized for communication. They are able to communicate with neurons and other cell types through ... from molecular and cellular studies of individual neurons to imaging of sensory and motor tasks in the brain. Neuroscience has ... These questions include the patterning and regionalization of the nervous system, neural stem cells, differentiation of neurons ... the cell bodies of the neurons containing the nucleus), and how neurotransmitters and electrical signals are used to process ...
... in affected neurons that cells cannot break down. Deregulation of the autophagy pathway and mutation of alleles regulating ... "The dynamic interaction of AMBRA1 with the dynein motor complex regulates mammalian autophagy". The Journal of Cell Biology ... Mechanism of cell death. Cells that undergo an extreme amount of stress experience cell death either through apoptosis or ... Mizushima N, Komatsu M (November 2011). "Autophagy: renovation of cells and tissues". Cell. 147 (4): 728-41. doi:10.1016/j.cell ...
Cell biologyEdit. Photomicrograph of the dorsal motor nucleus of the vagus nerve (DmX) in a transverse section along the upper ... Lewy neurites are abnormal neurites in diseased neurons, containing granular material and abnormal α-synuclein filaments ... Lewy bodies are abnormal aggregates of protein that develop inside nerve cells, contributing to Parkinson's disease (PD), the ... Neuromelanin-laden cells of the substantia nigra are visible in the background. Stains used: mouse monoclonal alpha-synuclein ...
Epigenetics of neurodegenerative diseases
Neurodengenerative diseases of motor neurons can cause degeneration of motor neurons involved in voluntary muscle control such ... Peripheral nervous system (PNS) diseases may be further categorized by the type of nerve cell (motor, sensory, or both) ... As SMN protein generally promotes the survival of motor neurons, mutations in SMN1 results in slow degeneration motor neurons ... is a motor neuron disease that involves neurogeneration. All skeletal muscles in the body are controlled by motor neurons that ...
As the null mice age, a marked loss of Purkinje cells in the cerebellum results in decreased motor coordination. However, this ... doi:10.1016/j.neuron.2004.09.017. PMID 15450159.. *^ Barco A, Bailey CH, Kandel ER (June 2006). "Common molecular mechanisms in ... cell cycle arrest. • learning or memory. • cellular copper ion homeostasis. • cellular response to copper ion. • cell cycle. • ... cell surface. • endoplasmic reticulum. • membrane raft. • anchored component of membrane. • extracellular exosome. • cell ...
Human digestive system
These include the various cells of the gastric glands, taste cells, pancreatic duct cells, enterocytes and microfold cells. ... The enteric nervous system consists of some one hundred million neurons that are embedded in the peritoneum, the lining of ... Another product is iron, which is used in the formation of new blood cells in the bone marrow. Medicine treats the spleen ... The parietal cells in the fundus of the stomach, produce a glycoprotein called intrinsic factor which is essential for the ...
Progressive supranuclear palsy
The affected brain cells are both neurons and glial cells. The neurons display neurofibrillary tangles (NFTs), which are clumps ... Patients with PSP usually seek or are referred to occupational therapy, speech-language pathology for motor speech changes ... There is some evidence that the hypnotic zolpidem may improve motor function and eye movements, but only from small-scale ... of tau protein, a normal part of a brain cell's internal structural skeleton. These tangles are often different from those seen ...
Upper motor neuron. *Lower motor neuron *α motorneuron. *β motorneuron. *γ motorneuron ... Changes in the white matter integrity of the corpus callosum may also be related to cognitive and motor function decline as ... Myelination: Schwann cell *Neurilemma. *Myelin incisure. *Node of Ranvier. *Internodal segment. *Satellite glial cell ...
Succinic semialdehyde dehydrogenase deficiency
... simultaneously releasing excitatory neurotransmitters onto motor neurons. Because the number and function of GABAB receptors ... Proper myelination is critical for carrying electrical signals, or data, from one nerve cell to the next. When myelin becomes ... MAP kinase is imperative for numerous physiological changes including regulation of cell division and differentiation, thus, ... motor, speech, and language as the most common manifestations. Later cases reported in the early 1990s began to show that ...
Brain-derived neurotrophic factor
negative regulation of neuron apoptotic process. • synapse assembly. • cell-cell signaling. • positive regulation of brain- ... motor neurons, the kidneys, saliva, and the prostate. BDNF itself is important for long-term memory. Although the vast ... regulation of neuron differentiation. • neuron projection morphogenesis. • modulation of chemical synaptic transmission. ... parts of the adult brain retain the ability to grow new neurons from neural stem cells in a process known as neurogenesis. ...
നാഡീവ്യൂഹം - വിക്കിപീഡിയ
... motor neuron) കൈമാറ്റം ചെയ്യുകയും, ചാലകന്യൂറോണുകൾ ഈ സന്ദേശങ്ങളെ പ്രവർത്തനക്ഷമമാകേണ്ട എഫക്ടർ കോശങ്ങളിൽ (effector cells) ... നാഡീകോശങ്ങൾക്കു പുറമേ ന്യൂറോലെമ്മ (Neurolemma), സാറ്റലൈറ്റ് കോശങ്ങൾ (satellite cells), ഗ്ലിയൽ കോശങ്ങൾ (glial cells), ... വിവിധതരം ബാഹ്യചോദനകൾ സംവേദക ന്യൂറോണുകൾ (sensory neuron) എന്ന സ്വീകരണ കോശങ്ങളിൽ എത്തിച്ചേരുന്നതോടെയാണ് ഈ പ്രവർത്തനശൃംഖല ... ഇവ പർക്കിൻജെ കോശങ്ങൾ (purkinje cells) എന്നറിയപ്പെടുന്നു. ശ്വേതദ്രവ്യത്തിൽ 4 ...
... which leads to cell death and in turn, affects the central nervous system and inhibits motor functions. The heart and other ... Demyelination is the loss of the myelin sheath around the axons of neurons, inhibiting their ability to communicate with other ... In the case of Leigh disease, crucial cells in the brain stem and basal ganglia are affected. This causes a chronic lack of ... Mitochondria are essential organelles in eukaryotic cells. Their function is to convert the potential energy of glucose, amino ...
Study investigates how to turn stem cells into motor neurons
A team of researchers investigates details of the cellular mechanisms involved in transforming a stem cell into a motor neuron ... Stem cells can develop into many different types of cells, such as muscle cells, red blood cells, or neurons. Given their ... Changing stem cells into motor neurons. Researchers uncovered a series of highly complex, independent changes that together ... A team of researchers uncovers new details involved in the process of turning stem cells into motor neurons. New research ...
Production of human motor neurons from stem cells is gaining speed | EurekAlert! Science News
... and thus produce different populations of motor neurons from these cells in only 14 days. This discovery, published in Nature ... leading to more rapid progress in understanding diseases of the motor system, such as infantile spinal amyotrophy or ... Biotechnology, will make it possible to expand the production process for these neurons, ... at I-Stem have recently developed a new approach to better control the differentiation of human pluripotent stem cells, ...
Neuroblastoma x spinal cord (NSC) hybrid cell lines resemble developing motor neurons. - PubMed - NCBI
Neuroblastoma x spinal cord (NSC) hybrid cell lines resemble developing motor neurons.. Cashman NR1, Durham HD, Blusztajn JK, ... NSC cell lines appear to model selected aspects of motor neuron development in an immortalized clonal system. ... a series of mouse-mouse neural hybrid cell lines by fusing the aminopterin-sensitive neuroblastoma N18TG2 with motor neuron- ... NSC-19, NSC-34, and their subclones express additional properties expected of motor neurons, including generation of action ...
Downregulation of VAPB expression in motor neurons derived from induced pluripotent stem cells of ALS8 patients. - PubMed -...
Motor neuron differentiation. (A) Expression of the motor neuron progenitor Islet-1 marker in neuroectodermal cells after 4 ... D) Live image of motor neuron-like cells expressing GFP under the control of the Hb9 promoter. Bar = 100 µm. (E) Control and (F ... Downregulation of VAPB expression in motor neurons derived from induced pluripotent stem cells of ALS8 patients.. Mitne-Neto M1 ... Downregulation of VAPB expression in motor neurons derived from induced pluripotent stem cells of ALS8 patients ...
Patente US20080206865 - Method of in vitro differentiation of neural stem cells, motor neurons and ... - Google Patentes
In one embodiment, the invention comprises culturing a population of cells comprising a majority of cells that are ... or RA wherein the cells are characterized by an neural tube-like rosette morphology and are Pax6+/Sox1+. ... A method of differentiating embryonic stem cells into neural and motor cells is disclosed. ... The functional motor neurons from the renewable source of hES cells offer generic human motor neurons for screening ...
Epigenetic Regulation of Motor Neuron Cell Death through DNA Methylation | Journal of Neuroscience
Nissl-stained sections of lumbar spinal cord sections were used to determine motor neuron cell body volume. Motor neurons were ... we evaluated the ability of NSC34 cells to differentiate into motor neuron-like cells. We compared NSC34 cells cultured for 5 d ... RG108 fostered hypertrophy of injured motor neurons but not contralateral motor neurons, as evidence by the increased cell body ... Dnmt3a immunoreactivity localizes to motor neurons (open arrows) and small non-neuronal cells. In control motor neurons (inset ...
Directed differentiation of human-induced pluripotent stem cells generates active motor neurons.
... motor neuron progenitors and motor neurons in regenerative medicine applications and in vitro modeling of motor neuron diseases ... motor neuron progenitors and motor neurons in regenerative medicine applications and in vitro modeling of motor neuron diseases ... human iPS cells could be differentiated to form motor neurons with a similar efficiency as hESCs. Human iPS-derived cells ... human iPS cells could be differentiated to form motor neurons with a similar efficiency as hESCs. Human iPS-derived cells ...
Human skin cells transformed directly into motor neurons
Motor neurons drive muscle contractions, and their damage underlies devastating ... ... working to develop new treatments for neurodegenerative diseases have been stymied by the inability to grow human motor neurons ... in this case motor neurons. In the future, we would like to study skin cells from patients with disorders of motor neurons. Our ... To convert skin cells into motor neurons, the researchers exposed the skin cells to molecular signals that are usually present ...
Motor neurone disease stem cell trial advances to next phase - BioNews
Motor neurone disease stem cell trial advances to next phase. 22 April 2013 ... A clinical trial to test the safety of a stem cell treatment for amyotrophic lateral sclerosis, the most common form of motor ... Reactivation of an ancient virus embedded in the human genome may trigger the onset of motor neurone disease.... ... ALS is a type of motor neuron disease often referred to as Lou Gehrigs and Maladie de Charcot. The late-onset condition, ...
Motor neurone disease: how could stem cells help? | Eurostemcell
A motor neuron that synapses onto another motor neuron within the CNS or a peripheral motor nerve within the PNS is sometimes ... cells that can make more of themselves and also any cell type in the body). The iPS cells were thus used to make motor neurons ... The researchers first transformed the skin cells into induced pluripotent stem cells (iPS cells)- iPS cells are cells that have ... ultimately leading to the degeneration of cells that form motor pathways - the motor neurons. Motor pathways generate and ...
Stem Cell-Derived Cranial and Spinal Motor Neurons Reveal Proteostatic Differences between ALS Resistant and Sensitive Motor...
... progressively degenerate while a subset of cranial motor neurons (CrMN) are spared until late ... Cell Metabolism (77) Cell Reports (708) Cell Reports Medicine (36) Cell Reports Physical Science (11) Cell Stem Cell (87) Cell ... Cell Press Journals on Sneak Peek All Journals (2686) Cancer Cell (46) Cell (289) Cell Chemical Biology (60) Cell Host & ... Stem Cell-Derived Cranial and Spinal Motor Neurons Reveal Proteostatic Differences between ALS Resistant and Sensitive Motor ...
Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial...
... which are involved in motor neuron death in ALS. Strikingly, skin-derived astrocytes show similar toxicity toward motor neurons ... We previously reported motor neuron toxicity through postmortem ALS spinal cord-derived astrocytes. However, these cells can ... Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial ... Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial ...
Pancreatic B Cell Function in Motor Neurone Disease | Clinical Science | Portland Press
Pancreatic B Cell Function in Motor Neurone Disease AJ Krentz AJ Krentz ... AJ Krentz, PM Clark, CN Hales, AC Williams, M Nattrass; Pancreatic B Cell Function in Motor Neurone Disease. Clin Sci (Lond) 1 ... Endocytosis and retrograde axonal traffic in motor neurons. Biochem Soc Symp (January, 2005) ...
Induced pluripotent stem cell derived motor neurons for the repair of acute cervical SCI
Target: Transplantation of iPS-cell derived neurons in combination with activation of the … ... Giles W. Plant, Lorry I lokey Stem Cell Building, , Stanford, USA Induced pluripotent stem cell derived motor neurons for the ... a) transplantation of specific cortical motor neurons derived from human iPS cells b) activation of the intrinsic growth ... Target: Transplantation of iPS-cell derived neurons in combination with activation of the intrinsic axon growth capacity (PTEN ...
HDAC6 Inhibitors Rescued the Defective Axonal Mitochondrial Movement in Motor Neurons Derived from the Induced Pluripotent Stem...
... and clinical studies in all areas of stem cell biology and applications. The journal will consider basic, translational, and ... Stem Cells International is a peer-reviewed, Open Access journal that publishes original research articles, review articles, ... CMT2F-motor neurons and dHMN2B-motor neurons also showed reduced -tubulin acetylation compared with controls. The newly ... tubulin and reversed axonal movement defects of mitochondria in CMT2F-motor neurons and dHMN2B-motor neurons. Our results ...
Stem cell study raises hope for those suffering from motor neuron disease | The Times & The Sunday Times
A landmark discovery using stem-cell technology could lead to the first clinical trials of drugs to defeat motor neuron disease ... Stem cell study raises hope for those suffering from motor neuron disease. ... Motor neuron disease, known as MND, is incurable, debilitating, and usually fast-acting, killing most sufferers relatively soon ... However, hopes of a treatment have risen in recent years as stem-cell science has provided the ability to model diseases in the ...
Human skin cells transformed directly into motor neurons - Washington University School of Medicine in St. Louis
... in this case motor neurons. In the future, we would like to study skin cells from patients with disorders of motor neurons. Our ... To convert skin cells into motor neurons, the researchers exposed the skin cells to molecular signals that are usually present ... And importantly, avoiding a stem cell state allows the resulting motor neurons to retain the age of the original skin cells and ... Louis have converted skin cells from healthy adults directly into motor neurons without going through a stem cell state. ...
Cell-based therapies for amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) | Cochrane
... including stem cell therapy. Stem cell therapy aims to provide new motor neurons, which may help stop or slow down disease ... Cell-based therapies for amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). Review question ... Abdul Wahid S, Law Z, Ismail N, Lai N. Cell-based therapies for amyotrophic lateral sclerosis/motor neuron disease. Cochrane ... Based on evidence from this trial, stem cell treatment may slightly reduce decline in motor function at six months, but may not ...
Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death...
Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death ... Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death ... Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death ... Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death ...
What Do You Call a Motor Neuron and All of the Skeletal Muscle Cells It Stimulates? | Reference.com
The University of Washington describes a motor unit as consisting of a somatic motor or efferent neuron and all the muscle ... fibers or cells it stimulates or innervates. An efferent axon reaches the... ... What Do You Call a Motor Neuron and All of the Skeletal Muscle Cells It Stimulates? ... of Washington describes a motor unit as consisting of a somatic motor or efferent neuron and all the muscle fibers or cells it ...
Nkx2.2 and Nkx2.9 Are the Key Regulators to Determine Cell Fate of Branchial and Visceral Motor Neurons in Caudal Hindbrain
... progenitors of branchiovisceral motor neurons in the ventral p3 domain of hindbrain are transformed to somatic motor neurons, ... Here we demonstrate that the formation of branchial and visceral motor neurons critically depends on the transcription factors ... and somatic motor neurons, which develop in typical patterns along the anteroposterior and dorsoventral axes of hindbrain. ... while the purely somatic hypoglossal and abducens motor nerves are not diminished. Cell lineage analysis in a genetically ...
Differentiation of Mouse Embryonic Stem Cells to Spinal Motor Neurons - Current Protocols
Controlled differentiation of embryonic stem (ES) cells into clinically relevant cell types is a fundamental goal of stem cell ... Basic Protocol 1: Differentiation of ES Cells into Motor Neurons. Materials * Dissociated ES cells suspended in ADFNK medium ( ... Human motor neuron differentiation from human embryonic stem cells. Stem Cells Dev. 14: 266‐269. ... Basic Protocol 1: Differentiation of ES Cells into Motor Neurons. *Support Protocol 1: Preparation of Embryonic Stem Cells for ...
Microphysiological 3D model of amyotrophic lateral sclerosis (ALS) from human iPS-derived muscle cells and optogenetic motor...
... from human iPS-derived muscle cells and optogenetic motor neurons Message Subject. (Your Name) has forwarded a page to you from ... from human iPS-derived muscle cells and optogenetic motor neurons ... human iPS-derived muscle cells and optogenetic motor neurons. ... from human iPS-derived muscle cells and optogenetic motor neurons ...
Functional Neuromuscular Junctions Formed by Embryonic Stem Cell-Derived Motor Neurons
Human embryonic stem cell-derived motor neurons expressing SOD1 mutants exhibit typical signs of motor neuron degeneration ... Conversion of mouse and human fibroblasts into functional spinal motor neurons. Cell stem cell. 2011;9:205-218. [PMC free ... Wichterle H, Peljto M. Differentiation of mouse embryonic stem cells to spinal motor neurons. Curr Protoc Stem Cell Biol. 2008; ... Directed Differentiation of Human-Induced Pluripotent Stem Cells Generates Active Motor Neurons. Stem Cells. 2009;27:806-811. [ ...
Stem cell technology offers new insight into motor neuron disease
... of Edinburgh have identified a specific type of cell in the human body which can cause motor neurons to fail using stem cell ... become damaging to motor neurons in motor neuron disease (MND).. By growing different combinations of glial cells and motor ... the negative influence of glial cells seems to prevent motor neurons from fulfilling their normal roles, even before the motor ... New stem cell technology reveals fresh insights into motor neuron disease More information: Chen Zhao et al. Mutant C9orf72 ...
Stem cell model of motor neuron disease wins International 3Rs Prize 2018 | NC3Rs
Functional and transcriptomic analysis showed that the cells have key aspects of ALS pathology including disrupted motor neuron ... The team used induced pluripotent stem cells (iPSCs) sourced from ALS patients to generate mature spinal cord motor neurons and ... Motor neuron disease also known as amyotrophic lateral sclerosis (ALS) affects around 5,000 adults a year in the UK and is ... synapse formation and mislocalisation of nuclear proteins leading to motor neuron death. By co-culturing different cell types, ...
Repair of Conus Medullaris/Cauda Equina Injury using Human ES Cell-Derived Motor Neurons | California's Stem Cell Agency
In this project, we grow human embryonic stem cells to develop into immature motor neurons, and these cells are transplanted ... We have shown that 1) Large numbers of motor neurons can be grown in tissue culture and later be used for cell transplantation ... The PI is planning to determine whether acute or delayed transplantation of human ES cell-derived motor and autonomic neurons ... Repair of Conus Medullaris/Cauda Equina Injury using Human ES Cell-Derived Motor Neurons ...
Involvement of neurofilaments in motor neuron disease | Journal of Cell Science
Involvement of neurofilaments in motor neuron disease Message Subject (Your Name) has sent you a message from Journal of Cell ... Motor neuron disease is clinically characterized by progressive muscle wasting leading to total muscle paralysis. A long ... These data indicate that extensive accumulation of neurofilaments in motor neurons can trigger a neurode-generative process and ... These transgenic mice show most of the hallmarks observed in motor neuron disease, including swollen perikarya with ...
Motor Neurons in Cu/Zn Superoxide Dismutase-Deficient Mice Develop Normally but Exhibit Enhanced Cell Death After Axonal Injury
... has focused much attention on the function of SOD1 as related to motor neuron survival. Here we describe the creation and ... These results indicate that Cu/Zn SOD is not necessary for normal motor neuron development and function but is required under ... However Cu/Zn SOD-deficient mice exhibit marked vulnerability to motor neuron loss after axonal injury. ... has focused much attention on the function of SOD1 as related to motor neuron survival. Here we describe the creation and ...
Stem cell model of motor neurone disease wins international 3Rs prize | Crick
Crick scientist Rickie Patani has been awarded an international prize for pioneering stem-cell techniques that reduce the need ... Motor neurons grown from iPS cells in the lab, viewed down a microscope. ... Then using chemical cues, they guide the stem cells into becoming motor neurones, or supporting cells, that can be studied in a ... Motor neuron disease, also known as amyotrophic lateral sclerosis (ALS), affects around 5,000 adults a year in the UK and is ...
DegenerationAtrophyUpper motor nWeaknessDifferentiationDiseaseDeath of motor neuronsDegeneration of motor neuronsNeural stemBrain and spinal cordPluripotentRetinoic acidResearchersProgenitorVitroGeneTypes of neuronsGenesGlialConvert skin cells into motor neuronsGenerateAstrocytesIPSCsRegenerativePathologyStem cell-derivedNervous systemHuman2017ISL1ProgenitorsSomaticSpinal motor neuronCortical motor neuronsFunctional motor neuronsInnervateSignalsSOD1Therapies
Upper motor n2
- Compressive lesions of the spinal cord usually cause a syndrome of upper motor neurone weakness, spasticity and sensory loss below the level of the lesion. (bmj.com)
- Compressive spinal cord lesions typically result in a combination of upper motor neurone weakness, spasticity, sensory loss and sphincter disturbance below the level of the lesion. (bmj.com)
- It has long been recognised that compressive cervical cord lesions may present as isolated lower motor neurone weakness of the upper limbs, a syndrome termed cervical spondylotic amyotrophy. (bmj.com)
- We describe two patients presenting with isolated lower motor neurone weakness of the lower limbs in association with a compressive cord lesion at T11/12, a condition we have termed thoracic spondylotic amyotrophy. (bmj.com)
- Isolated lower motor neurone weakness affecting the upper limbs due to a compressive cervical cord lesion was first reported by Brain and colleagues 1 in 1952 and has since been termed cervical spondylotic amyotrophy. (bmj.com)
- 1 We describe two patients presenting with slowly progressive isolated lower motor neurone weakness affecting the lower limbs in association with a compressive spinal cord lesion at T11/12. (bmj.com)
- Researchers at the Institute for Stem Cell Therapy and Exploration of Monogenic Diseases (I-Stem - Inserm/AFM/UEVE), in collaboration with CNRS and Paris Descartes University, have recently developed a new approach to better control the differentiation of human pluripotent stem cells, and thus produce different populations of motor neurons from these cells in only 14 days. (eurekalert.org)
- This inefficiency is partly due to a poor understanding of the molecular mechanisms controlling the differentiation of these cells. (eurekalert.org)
- Inserm researchers at the Institute for Stem Cell Therapy and Exploration of Monogenic Diseases (I-Stem - Inserm/French Muscular Dystrophy Association [AFM]/University of Évry Val d'Essonne [UEVE]), in collaboration with CNRS and Paris-Descartes University, have developed an innovative approach to study the differentiation of human stem cells and thus produce many types of cells in an optimal manner. (eurekalert.org)
- The targeted differentiation of human pluripotent stem cells is often a long and rather inefficient process. (eurekalert.org)
- The in vitro differentiation of ES cells provides new perspectives for studying the cellular and molecular mechanisms of early development and the generation of donor cells for transplantation therapies. (google.es)
- Directed differentiation of human-induced pluripotent stem cells generates active motor neurons. (ca.gov)
- The potential for directed differentiation of human-induced pluripotent stem (iPS) cells to functional postmitotic neuronal phenotypes is unknown. (ca.gov)
- In addition to its role in protein refolding, HSPB1 is involved in protein translation, intracellular reduction/oxidation state, cytoskeletal structure, cell differentiation, and apoptosis [ 6 ]. (hindawi.com)
- Differentiation strategy for motor neurogenesis and astrogliogenesis (derived from Figure 1 of Hall et al. (nc3rs.org.uk)
- Continued and ongoing analysis will further characterize the survival and differentiation pattern of transplanted human motor neurons as well as the effects on functional and anatomical outcome measures. (ca.gov)
- The robust iPSC differentiation protocols developed by the team and laid out in a Cell Reports publication recognised by the award is a good example of a transition to animal free innovations. (crick.ac.uk)
- SCOTCH PLAINS, NJ - April 27, 2006 - Stem Cell Innovations, Inc. (OTCBB: SCLL) has filed a patent application protecting the differentiation of its proprietary human pluripotent stem cells (PCs) into neural progenitor cells and purified populations of motor neurons. (vb.is)
- MN embryoid bodies (MEBs) were grown in these MEA chips, and differentiation of mES cells into MNs was monitored by the expression of eGFP with a MN specific promoter, Hb9. (illinois.edu)
- The in vitro differentiation of embryonic stem cells (ESCs) provides new perspectives for studying the cellular and molecular mechanisms of early development and the generation of donor cells for transplantation therapies. (justia.com)
- Here we show a near complete restriction of hESCs to ventral spinal progenitors (Olig2+, NKX2.2+, Irx3+/Pax7−) and efficient differentiation of motor neurons (HB9+) by a simple sequential application of retinoid acid (RA), and sonic hedgehog (SHH) in a chemically defined condition. (justia.com)
- Directed differentiation of human pluripotent stem cells - human embryonic stem cells (hESCs) and human induced pluripotent stem cells (hiPSCs) - toward specific lineages is the first crucial step in order to extensively employ these cells in early human development investigation and potential clinical applications. (biomedcentral.com)
- This protocol describes the differentiation of a transgenic GFP reporter cell line into motor neuron cells. (lifemapsc.com)
- Pluripotent stem cells can be genetically labeled to facilitate differentiation studies. (elsevier.com)
- In this paper, we describe a gene-targeting protocol to knock in a GFP cassette into key gene loci in human pluripotent stem cells (hPSCs), and then use the genetically tagged hPSCs to guide in vitro differentiation, immunocytochemical and electrophysiological profiling and in vivo characterization after cell transplantation. (elsevier.com)
- Treatment with GSK3β inhibitors during the initial phase of differentiation in combination with dual SMAD inhibition was sufficient to induce PAX6 + and SOX1 + neural progenitors within 1 week, and subsequent treatment with retinoic acid (RA) and purmorphamine, which activates sonic hedgehog (SHH) signaling, resulted in the highly efficient induction of HB9 + and ISL-1 + motor neurons within 2 weeks. (biomedcentral.com)
- After 4 weeks of monolayer differentiation in motor neuron maturation medium, hPSC-derived motor neurons were shown to mature, displaying larger somas and clearer staining for the mature motor neuron marker choline acetyltransferase (ChAT). (biomedcentral.com)
- Our motor neuron differentiation system and lentivirus-based reporter system for motor neurons facilitate the analysis of disease-specific hiPSCs for motor neuron diseases. (biomedcentral.com)
- Scientists are unable to properly innervate motor neurons in a mouse model, so what they did was knockdown a gene in mice that controls motor neuron differentiation which caused the spinal cord to revert back to a primitive state. (sophiascure.org)
- We believe that the studies will not only elucidate the complex, yet highly reproducible processes of cell differentiation during development but also provide new and powerful tools for modeling, studying and treating human motor neuron diseases in the near future. (nyu.edu)
- Here we will review methods and molecular factors for directed differentiation of stem cells into spinal motor neurons, the potential uses of these models for dissecting the mechanisms underlying glia-induced motor neuron degeneration and screening for new therapeutics aimed at protecting motor neurons in ALS, as well as discuss challenges facing the development of motor neuron replacement-based cell therapies for recovery in ALS. (stemcellrevolution.com)
- The recombinant form of this protein was shown to promote the survival and differentiation of dopaminergic neurons in culture, and was able to prevent apoptosis of motor neurons induced by axotomy. (wikipedia.org)
- Our conversion process should model late-onset aspects of the disease using neurons derived from patients with the condition. (medicalxpress.com)
- The ability of scientists to convert human skin cells into other cell types, such as neurons, has the potential to enhance understanding of disease and lead to finding new ways to heal damaged tissues and organs, a field called regenerative medicine. (medicalxpress.com)
- ALS, also known as Lou Gehrig's disease, is a late onset neurodegenerative disease where nerve cells that control voluntary muscle movements are damaged, leading to paralysis in those affected. (bionews.org.uk)
- Reactivation of an ancient virus embedded in the human genome may trigger the onset of motor neurone disease. (bionews.org.uk)
- The US Food and Drug Administration (FDA) has awarded 'fast-track' status to a potential stem cell therapy for Amyotrophic Lateral Sclerosis (ALS), or motor neurone disease. (bionews.org.uk)
- A clinical trial to test the safety of a stem cell treatment for amyotrophic lateral sclerosis, the most common form of motor neurone disease, suggests that the new therapy is safe and well-tolerated. (bionews.org.uk)
- Motor neurone disease: how could stem cells help? (eurostemcell.org)
- Motor neurone disease (MND) is a rare but debilitating and ultimately fatal neurodegenerative disorder. (eurostemcell.org)
- Several different conditions caused by damage to nerve cells (neurons) controlling our muscles are collectively classified as Motor Neurone Disease (MND). (eurostemcell.org)
- In amyotrophic lateral sclerosis (ALS) spinal motor neurons (SpMN) progressively degenerate while a subset of cranial motor neurons (CrMN) are spared until late stages of the disease. (ssrn.com)
- The Charcot-Marie-Tooth disease 2F (CMT2F) and distal hereditary motor neuropathy 2B (dHMN2B) are caused by autosomal dominantly inherited mutations of the heat shock 27 kDa protein 1 ( HSPB1 ) gene and there are no specific therapies available yet. (hindawi.com)
- The absolute velocity of mitochondrial movements and the percentage of moving mitochondria in axons were lower both in CMT2F-motor neurons and in dHMN2B-motor neurons than those in controls, and the severity of the defective mitochondrial movement was different between the two disease models. (hindawi.com)
- A landmark discovery using stem-cell technology could lead to the first clinical trials of drugs to defeat motor neuron disease within "five to ten years", according to British-led research. (thetimes.co.uk)
- Similar skin samples from patients with neurodegenerative diseases could allow scientists to study the disease in its native cell type. (wustl.edu)
- also known as motor neuron disease or MND) is a condition in which nerves in the brain and spinal cord that control movement (motor neurons) stop working. (cochrane.org)
- Cell-based therapy can be defined as injection of cellular material into a person to treat disease. (cochrane.org)
- Stem cell therapy aims to provide new motor neurons, which may help stop or slow down disease progression in people with ALS/MND. (cochrane.org)
- Previous reviews supported the use of cell-based therapy as a potential means of delaying the disease course in ALS/MND, but these were mainly based on preclinical animal models. (cochrane.org)
- Proximal spinal muscular atrophy is an autosomal recessive human disease of spinal motor neurons leading to muscular weakness with onset predominantly in infancy and childhood. (pnas.org)
- A key objective of stem cell biology is to create physiologically relevant cells suitable for modeling disease pathologies in vitro. (pubmedcentralcanada.ca)
- Much progress towards this goal has been made in the area of motor neuron (MN) disease through the development of methods to direct spinal MN formation from both embryonic and induced pluripotent stem cells. (pubmedcentralcanada.ca)
- Over the past decade, considerable attention has been focused on using stem cell-derived MNs to model disease pathogenesis, driven by demonstrations that mouse and human embryonic stem cells (mESCs and hESCs) can be directed to form MNs in response to developmental signals that promote MN formation in vivo - . (pubmedcentralcanada.ca)
- The joint research, published today in Glia , has shown that glial cells , which normally support neurons throughout the brain and spinal cord, become damaging to motor neurons in motor neuron disease (MND). (medicalxpress.com)
- He said: "We are very excited by these new findings which clearly point the finger at glial cells as key players in this devastating disease. (medicalxpress.com)
- MND is a progressive neurodegenerative disease that leads to paralysis because it attacks motor neurons , a special type of nerve cell in the brain and spinal cord that generates the electrical signals needed to control all of our movements. (medicalxpress.com)
- A paper describing the use of patient-derived stem cells to study motor neuron disease has been recognised with the annual £30k prize awarded by the NC3Rs and sponsored by GSK. (nc3rs.org.uk)
- Motor neuron disease also known as amyotrophic lateral sclerosis (ALS) affects around 5,000 adults a year in the UK and is characterised by a progressive degeneration of motor neurons in the brain and spinal cord that ultimately leads to death. (nc3rs.org.uk)
- By co-culturing different cell types, the team demonstrated that astrocytes have an active pathogenic role in the disease which further perturbs the survival of motor neurons. (nc3rs.org.uk)
- The cells provide a tool to better understand the disease and to screen potential therapeutics, allowing some studies which are currently dependent on mouse models to be carried out in vitro . (nc3rs.org.uk)
- Motor neuron disease is clinically characterized by progressive muscle wasting leading to total muscle paralysis. (biologists.org)
- In addition to the wide-spread loss of these neurons, neuronal abnormalities including massive accumulation of neurofilaments in cell bodies and proximal axons have been also widely observed, particularly in the early stages of the disease. (biologists.org)
- These transgenic mice show most of the hallmarks observed in motor neuron disease, including swollen perikarya with eccentrically localized nuclei, proximal axonal swellings, axonal degeneration and severe skeletal muscle atrophy. (biologists.org)
- Marian Blanca Ramírez from the CSIC in Spain has been studying the effects of LRRK2, a protein associated with Parkinson's disease, on cell motility. (biologists.org)
- Crick scientist Rickie Patani has been awarded an international prize for pioneering stem-cell techniques that reduce the need for animals in motor neurone disease research. (crick.ac.uk)
- The prize, awarded by the National Centre for the Replacement, Refinement & Reduction of Animals in Research (NC3Rs) and sponsored by GSK , recognizes Rickie's use of patient-derived stem cells to better understand the disease and screen potential therapeutics without relying on animal models. (crick.ac.uk)
- This so-called 'induced pluripotent stem cell' (iPSC) approach has several scientific benefits over mouse models, including the ability to reflect the genetic diversity of patients, investigate sporadic forms of the disease (which represent 90% of the cases), and allow early molecular events to be studied. (crick.ac.uk)
- Various degenerative disease, immune disorders, injuries, and age-related conditions are showing therapeutic benefit from stem cell treatments. (placidway.com)
- If you are seeking treatment of a disease or looking to slow down the aging process, we can help you with stem cell therapy. (placidway.com)
- Clinical trials for stem cell transplants to grow a new immune system among people suffering from Crohn's disease has started. (placidway.com)
- If you are looking for Crohn disease treatment using stem cell therapy abroad, you should visit Los Algodones, Mexico. (placidway.com)
- PlacidWay Medical Tourism provides information about Stem Cell Therapy for Motor Neuron Disease MND options abroad to patients from around the world. (placidway.com)
- Amyotrophic Lateral Sclerosis is a progressively lethal motor neuron disease with no known cure. (jefferson.edu)
- We further analyzed how FUS-induced alterations in astrocytes alter communication between astrocytes and two cell-types highly relevant to neurodegenerative disease: motor neurons and endothelial cells. (jefferson.edu)
- Taken together these studies extend evidence from different genetic models of ALS that astrocytes contribute to disease, provide novel findings that FUS-ALS astrocytes induce pathogenic changes to motor neurons and endothelial cells of the blood-brain-barrier, and provide new mechanistic insights into these phenomena which warrant future studies. (jefferson.edu)
- As her understanding of these survival pathways grew, Dr McConnell realised that cancer stem cells might just hold the secret to extending the life of cells that die prematurely, such as in motor neurone disease. (malaghan.org.nz)
- Patients with motor neurone disease suffer increasing weakness of the muscles, due to the death of the neurons that feed into them and there is currently very little that can be done to stop this. (malaghan.org.nz)
- Are you searching for the best package for Parkinson Disease live cell treatment? (placidway.com)
- Get the information on the best and most affordable live cell treatment for Parkinson disease in Germany. (placidway.com)
- SMN-dependent mis-spliced transcripts in motor neurons may cause stresses that are particularly harmful and may serve as potential targets for the treatment of motor neuron disease or as biomarkers in the SMA patient population. (iupui.edu)
- Raju, Trichur 2018-06-01 00:00:00 Disease modeling has become challenging in the context of amyotrophic lateral sclerosis (ALS), as obtaining viable spinal motor neurons from postmortem patient tissue is an unlikely possibility. (deepdyve.com)
- My lab employs motor neurons generated from mouse and human embryonic (ES) and induced pluripotent (iPS) stem cells, as well as mouse animal models to investigate motor neuron development and disease. (labroots.com)
- To identify ncRNAs participated during motor neuron development and degeneration, we used human embryonic stem cell and motor neuron disease iPSCs as paradigms and robustly harnessed them into different motor neuron subtypes to perform strand specific RNA-seq and small RNA-seq simultaneously. (labroots.com)
- Preparation of high-quality RNA from cells of interest is critical to precise and meaningful analysis of transcriptional differences among cell types or between the same cell type in health and disease or following pharmacologic treatments. (leica-microsystems.com)
- Mrs Terpstra, 64, was diagnosed with the degenerative condition motor neurone disease about a year ago. (thisisms.com)
- Scientists have discovered a new way to generate human motor nerve cells in a development that will help research into motor neurone disease. (esciencenews.com)
- Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of motor neurons (MNs). (elsevier.com)
- Chen, K , Northington, F & Martin, LJ 2010, ' Inducible nitric oxide synthase is present in motor neuron mitochondria and Schwann cells and contributes to disease mechanisms in ALS mice ', Brain Structure and Function , vol. 214, no. 2-3, pp. 219-234. (elsevier.com)
- Stem cell-derived motor neurons represent a promising research tool in disease modeling, drug screening, and development of therapeutic approaches for MNDs and spinal cord injuries. (biomedcentral.com)
- MNDs can be classified in relation to the subpopulation of MNs affected mainly by the disease process as spinal muscular atrophy (SMA), progressive muscular atrophy, spinobulbar muscular atrophy (or Kennedy's disease), and hereditary motor neuropathies involving lower MNs. (biomedcentral.com)
- SMA is an autosomal recessive disease: the majority of patients with SMA carry mutations in the SMN1 gene (survival motor neuron 1), resulting in the selective degeneration of lower α-MNs. (biomedcentral.com)
- Ultrastructural study of the synapses of central chromatolytic anterior horn cells in motor neuron disease. (semanticscholar.org)
- This report deals with an ultrastructural investigation of the synapses on the somata of central chromatolytic anterior horn neurons of seven patients with amyotrophic lateral sclerosis (ALS) and four patients with lower motor neuron disease (LMND) who had no upper motor neuron or corticospinal tract involvement. (semanticscholar.org)
- When these nerve cells are damaged, or stop working properly, Motor Neurone Disease occurs. (globalstemcells.com)
- When the disease progresses, which is known as neurodegeneration, people with Motor Neuron Disease find some or all of these activities increasingly difficult. (globalstemcells.com)
- There's no single test to diagnose Motor Neurone Disease and diagnosis is based mainly on the opinion of a neurologist. (globalstemcells.com)
- Japanese stem cell scientists have succeeded in slowing the deterioration of mice with motor neuron disease, possibly paving the way for human treatment, according to a new paper. (stem-cells-news.com)
- A team of researchers from the Kyoto University and Keio University transplanted specially created cells into mice with amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's, or motor neuron disease. (stem-cells-news.com)
- Advancements in stem cell research may one day help surgeons provide treatment for Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's disease, a deadly neurodegenerative disease characterized by the selective death of motor neurons. (stem-cells-news.com)
- A team of Harvard stem cell researchers has succeeded in reprogramming adult mouse skin cells directly into the type of motor neurons damaged in amyotrophic lateral sclerosis (ALS), best known as Lou Gehrig's disease, and spinal muscular atrophy (SMA). (stem-cells-news.com)
- Neuralstem Inc. has received the green light to begin the first human stem cell trial to treat Amyotrophic Lateral Sclerosis (ALS), often referred to as Lou Gehrig's disease. (stem-cells-news.com)
- In particular, neural cells derived from disease-specific human induced pluripotent stem cells (hiPSCs) established from patients with neurological disorders have been used as in vitro disease models to recapitulate in vivo pathogenesis because neural cells cannot be usually obtained from patients themselves. (biomedcentral.com)
- In particular, neural cells derived from disease-specific hiPSCs from patients with neurological disorders have been especially useful as in vitro disease models recapitulating in vivo pathogenesis, as cells in the nervous system cannot be usually obtained from patients themselves. (biomedcentral.com)
- This method facilitates simple and accurate pathophysiological analysis of motor neuron diseases using disease-specific hiPSCs. (biomedcentral.com)
- Despite intensive research, there is still no known cure or standard treatment for Amyotrophic Lateral Sclerosis (ALS), a Motor Neuron Disease (MND) subtype. (axolbio.com)
- Motor Neurone Disease Awareness Month takes place in June of every year. (globalstemcells.com)
- Its purpose is to help raise awareness of Motor Neurone Disease (MND), which is a group of diseases that affect the nerve cells, especially motor neurons in the brain and spinal cord. (globalstemcells.com)
- One of the most commonly known example of Motor Neurone Disease is Amyotrophic Lateral Sclerosis (ALS) , or Lou Gehrig's Disease, which is named after a baseball player who died from this disease in 1941. (globalstemcells.com)
- The theme for MND Awareness this year is a continuation from last year's motto: "But With Motor Neurone Disease, My Eyes Could Be All I Can Move. (globalstemcells.com)
- To help patients with Motor Neurone Disease, globalstemcells.com and Unique Access Medical provide access to a progressive and responsible treatment , which is aimed to increase our patients' quality of lives. (globalstemcells.com)
- This causes a disorder which is predominantly a motor neuron disease,' Sendtner said. (alsresearchforum.org)
- Watching how stem cells (derived from ALS patients' own skin cells, for example) develop into motor neurons will offer new insights into disease processes, and any method that improves the speed and efficiency of generating the motor neurons will aid scientists. (blogspot.com)
- Professor Siddharthan Chandran is Director of the Euan MacDonald Centre for Motor Neurone Disease Research at the University of Edinburgh. (alsnewstoday.com)
- Although isolated lower motor neuron disease has been reported as a paraneoplastic complication, it has not been previously described in association with anti-Hu antibody. (elsevier.com)
- Death from the complications of motor neuron disease ensued 23 months after the onset of weakness. (elsevier.com)
- Despite the absence of symptomatic cerebellar disease, a decrease in the number of Purkinje cells was also detected. (elsevier.com)
- Stem cells provide novel sources of cell therapies for motor neuron disease that have recently entered clinical trials. (elsevier.com)
- Transplantation was done prior to the onset of motor neuron disease. (elsevier.com)
- Our results support the potential of NSC grafts at multiple levels of spinal cord as future cellular therapy for motor neuron disease. (elsevier.com)
- Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease caused by the selective loss of both spinal and upper motor neurons. (stemcellrevolution.com)
- Motor neurone disease (MND), also known as amyotrophic lateral sclerosis, is a condition in which patients lose motor independence. (evolving-science.com)
- MND has been found to be associated with characteristic changes in the mitochondrial function, metabolism and overall growth of the nerve cells at the centre of motor function loss in the disease. (evolving-science.com)
- Therefore, Elbasiouny and his team conducted two- and three-dimensional studies of alpha -motor neurones in SOD1-G93A mice at various stages of their life-spans and disease progression. (evolving-science.com)
- U.S., March 6 -- ClinicalTrials.gov registry received information related to the study (NCT03067857) titled 'Autologous Bone Marrow-Derived Stem Cell Therapy for Motor Neuron Disease' on Feb. 25. (shine.com)
- Study Start Date: September 2016 Study Type: Interventional Condition: Motor Neuron Disease Amyotrophic Lateral Sclerosis Primary Lateral Sclerosis Progressive Muscular Atrophy Progressive Bulbar Palsies Intervention: Biological: Stem Cells Autologous bone marrow-derived stem cell populations injected via the intravenous and intrathecal routes. (shine.com)
- Stem cell-derived motor neurons could be used to repair the diseased or damaged spinal cord and to study neurodegenerative diseases such as amyotrophic lateral sclerosis (also known as Lou Gehrig's disease) and spinal muscular atrophy. (ucla.edu)
- The research was funded by the National Institute for Neurological Disease and Stroke, the March of Dimes Foundation, the Whitehall Foundation and the UCLA Broad Stem Cell Research Center. (ucla.edu)
- Motor Neurone Disease - Now contains new research into stem cell treatment. (hubpages.com)
- Spinal muscular atrophy (SMA) is a motor neuron disease and the leading genetic cause of death among infants and toddlers. (news-medical.net)
- Characterized by selective loss of nerve cells in the spinal cord, the disease leads to increasing muscular weakness and atrophy. (news-medical.net)
- GDNF has regenerative properties for brain cells and showed potential as treatment for Parkinson's disease - monkeys with an induced form of Parkinson's disease showed less trembling when treated with the drug, and neuronal fibres grew in part of the human brain exposed to the drug. (wikipedia.org)
Death of motor neurons2
- Rapid access to large quantities of neurons will be useful for testing a significant number of pharmacological drugs in order to identify those capable of preventing the death of motor neurons," concludes Cécile Martinat. (eurekalert.org)
- All these changes along with the enhanced expression of pro-apoptotic proteins-Bax and caspase 9-culminated in the death of motor neurons. (deepdyve.com)
Degeneration of motor neurons1
- Through epigenetic reprogramming techniques - first introduced in 2006 by Nobel Prize winner Shinya Yamanaka and colleagues - these cells have been artificially transformed into neural stem cells. (medicalnewstoday.com)
- This process involved transforming fibroblasts - a type of cell found in connective tissue - first into pluripotent cells, then into neural stem cells, and finally into neurons. (medicalnewstoday.com)
- Up to 15 patients with ALS will be enrolled to receive injections of neural stem cells derived by US company NeuralStem from the spinal cord of an eight-week-old aborted fetus . (bionews.org.uk)
- The next stage of the trials, which will take place at both the Emory ALS Center , as well as the University of Michigan , is designed to test the efficacy of the neural stem cells as a treatment for ALS, while still constantly monitoring safety aspects. (bionews.org.uk)
- In the present study, we transplanted human neural stem cells (NSCs) into the ventral horn of both the lumbar (L4-L5) and cervical (C4-C5) protuberance of SOD1 G93A rats, in an effort to test the feasibility and general efficacy of a dual grafting paradigm addressing several muscle groups in the front limbs, hind limbs and the respiratory apparatus. (elsevier.com)
- Neural stem cells (NSCs) have some specified properties but are generally uncommitted and so can change their fate after exposure to environmental cues. (utmb.edu)
Brain and spinal cord3
- Motor pathways generate and control movement by relaying electrical signals from brain and spinal cord motor neurons to the muscles via synapses (connections) with peripheral motor neurons. (eurostemcell.org)
- Neurons are the primary components of the central nervous system, which includes the brain and spinal cord, and of the peripheral nervous system, which comprises the autonomic nervous system and the somatic nervous system. (wikipedia.org)
- Motor neurons receive signals from the brain and spinal cord to cause everything from muscle contractions and affect glandular outputs. (wikipedia.org)
- Human pluripotent stem cells have the ability to give rise to every cell in the body. (eurekalert.org)
- However, the development and realisation of these clinical applications is often limited by the inability to obtain specialised cells such as motor neurons from human pluripotent stem cells in an efficient and targeted manner. (eurekalert.org)
- Downregulation of VAPB expression in motor neurons derived from induced pluripotent stem cells of ALS8 patients. (nih.gov)
- Fibroblasts from ALS8 patients and their non-carrier siblings were successfully reprogrammed to a pluripotent state and differentiated into motor neurons. (nih.gov)
- Human embryonic stem (ES) cells are pluripotent cells derived from the inner cell mass of pre-implantation embryos (Thomson, J. A., et al. (google.es)
- Avoiding the stem cell phase eliminates ethical concerns raised when producing what are called pluripotent stem cells , which are similar to embryonic stem cells in their ability to become all adult cell types. (medicalxpress.com)
- Going back through a pluripotent stem cell phase is a bit like demolishing a house and building a new one from the ground up," Yoo said. (medicalxpress.com)
- Here, we assessed the potential therapeutic effect of HDAC6 inhibitors on peripheral neuropathy with HSPB1 mutation using in vitro model of motor neurons derived from induced pluripotent stem cells (iPSCs) of CMT2F and dHMN2B patients. (hindawi.com)
- The team used induced pluripotent stem cells (iPSCs) sourced from ALS patients to generate mature spinal cord motor neurons and astrocytes. (nc3rs.org.uk)
- I'm honoured and grateful to receive this award for our efforts to develop human induced pluripotent stem cell models of neurodegeneration. (nc3rs.org.uk)
- SCI has developed and protected a unique pluripotent stem cell called the PC(TM). (vb.is)
- Specification of distinct cell types from hESCs is key to the potential application of these naïve pluripotent cells in regenerative medicine. (justia.com)
- Induced pluripotent stem cells (iPSCs) can be generated from patients' own somatic cells (for example, fibroblasts) by reprogramming them with specific factors. (biomedcentral.com)
- Dr. Svendsen's lab begins the processes of transforming the blood cells, known as peripheral blood mononuclear cells (PBMCs), into induced pluripotent stem (iPS) cells. (answerals.org)
- IPSCs are a type of pluripotent stem cell (meaning a type of stem cell that can become any of the cell types that make up the body) that are generated directly from adult blood cells (not embryonic cells). (answerals.org)
- The iPSC technology was pioneered by Shinya Yamanaka's lab in Kyoto, Japan, who showed in 2006 that the introduction of four specific genes encoding transcription factors could convert adult cells into pluripotent stem cells. (answerals.org)
- Pluripotent stem cells are an excellent tool to help study ALS because they can propagate indefinitely (therefore providing researchers with plenty of material to study) and are derived from individual patients. (answerals.org)
- Human pluripotent stem cells (hPSCs) are being applied in regenerative medicine and for the in vitro modeling of human intractable disorders. (biomedcentral.com)
- Human pluripotent stem cells (hPSCs), including human embryonic stem cells (hESCs) and human induced pluripotent stem cells (hiPSCs), can be differentiated into all cell types of the human body and have been applied in regenerative medicine and the pathophysiological analysis of intractable disorders [ 1 ]. (biomedcentral.com)
- This is one of our most important research aims here at Axol, where we have leveraged advances in stem cell biology to develop motor neurons derived from human induced pluripotent stem cells (hiPSCs) . (axolbio.com)
- Access to a virtually unlimited supply of spinal motor neurons creates a unique opportunity to decipher molecular processes governing the conversion of a pluripotent stem cell to a committed and differentiated cell type at global and comprehensive level. (nyu.edu)
- A technique that requires stem cell state often raises ethical issues, as is the case with pluripotent stem cells. (smanewstoday.com)
- Going back through a pluripotent stem cell phase is a bit like demolishing a house and building a new one from the ground up," Andrew S. Yoo , the study's lead author and an assistant professor of developmental biology at Washington University, said in a press release . (smanewstoday.com)
- Retinoic acid promotes neural and represses mesodermal gene expression in mouse embryonic stem cells in culture. (currentprotocols.com)
- In this study, human embryonic stem cells-BJNHem20-were differentiated into motor neurons expressing HB9, Islet1, and choline acetyl transferase using retinoic acid and purmorphamine. (deepdyve.com)
- ESMN was generated by exposing mouse ES cells to retinoic acid and sonic hedgehog. (ac.ir)
- In one embodiment, the invention comprises culturing a population of cells comprising a majority of cells that are characterized by an early rosette morphology and are Sox1−/Pax6+ in the presence of retinoic acid, wherein the cells express Hoxb4, but not Otx2 or Bf1. (justia.com)
- Other recommended reagents to support motor neuron progenitor culture include retinoic acid, Brain-derived Neurotrophic Factor, Ciliary Neurotrophic Factor and ROCK inhibitor (Y-27632 2HCl). (axolbio.com)
- A team of researchers uncovers new details involved in the process of turning stem cells into motor neurons. (medicalnewstoday.com)
- New research uncovers details of the transformation process that could one day enable researchers to create new types of cells. (medicalnewstoday.com)
- The researchers analyzed the changes that occur in the cells during the direct reprogramming process. (medicalnewstoday.com)
- Unlike commonly studied mouse motor neurons, human motor neurons growing in the lab would be a new tool since researchers can't take samples of these neurons from living people but can easily take skin samples. (medicalxpress.com)
- To convert skin cells into motor neurons, the researchers exposed the skin cells to molecular signals that are usually present at high levels in the brain. (medicalxpress.com)
- In the new study, the researchers extensively characterized this repackaging process, detailing how skin cells reprogrammed into generic neurons then can be guided into specific types of neurons. (medicalxpress.com)
- After much experimentation with multiple combinations, the researchers found that adding two more signals to the mix-transcription factors called ISL1 and LHX3-turned the skin cells into spinal cord motor neurons in about 30 days. (medicalxpress.com)
- Researchers are using stem cells to develop methods for growing neurons and other cells in a dish as a way to recreate (or 'model') MND. (eurostemcell.org)
- This greatly helps researchers study and discover the causes of neuron damage and what might prevent it. (eurostemcell.org)
- Researchers are examining ways stem cells might be used to repair the nervous system by growing new neurons and supporting cells. (eurostemcell.org)
- By growing different combinations of glial cells and motor neurons together in the lab, the researchers found that glial cells from patients can cause motor neurons from healthy individuals to stop producing the electrical signals they need to control muscles. (medicalxpress.com)
- The researchers used stem cells derived from patient skin samples to understand why MND causes motor neurons to lose their ability to generate the electrical signals needed to control muscles. (medicalxpress.com)
- First Person is a series of interviews with the first authors of a selection of papers published in Journal of Cell Science, helping early-career researchers promote themselves alongside their papers. (biologists.org)
- The researchers were first interested in how neural cells would differentiate in the absence of a certain methyltransferase called DNMT3A. (nyscf.org)
- But in earlier studies, the same researchers successfully used embryonic stem cells from mice and turned them into motor neurons. (globalstemcells.com)
- The efficiency of this embryonic method on mice has given the researchers details of the mechanisms taking place within cells. (globalstemcells.com)
- Researchers from the University of Washington School of Medicine in St. Louis have transformed skin cells from healthy adults directly into motor neurons without going through a stem cell state. (mims.com)
- Researchers have discovered a way to refine the production of retinal cells from embryonic stem cells for treating blindness in the elderly. (stem-cells-news.com)
- In an effort to identify the underlying causes of neurological disorders that impair motor functions such as walking and breathing, UCLA researchers have developed a novel system to measure communication between stem cell-derived motor neurons and muscle cells in a Petri dish. (stem-cells-news.com)
- The researchers also used optogenetics to engineer cells (both motor neurons and muscle cells) that would activate in response to certain wavelengths of light. (alsnewstoday.com)
- After numerous tests to confirm that their device worked as intended and formed functional junctions, the researchers used their device to examine how cell activity affected neural regrowth after injury. (alsnewstoday.com)
- The researchers examined the expression of several molecular factors known to be involved in motor neuron growth. (alsnewstoday.com)
- T]his study suggests the previously undescribed role of muscular activity through a paracrine mechanism to promote axonal regrowth of MNs [motor neurons] after injury," the researchers wrote. (alsnewstoday.com)
- The researchers studied the CNTF pathway in primary motor neuron cultures from embryonic PMN mice. (alsresearchforum.org)
- Researchers report they can generate human motor neurons from stem cells much more quickly and efficiently than previous methods allowed. (blogspot.com)
- Connectomics' allows researchers to identify all neurons in a given brain region, as well as map all of their connections. (biologists.org)
- Researchers at the Washington University School of Medicine in St. Louis, Missouri, were able to convert skin cells from healthy adults into motor neurons. (smanewstoday.com)
- Future studies with neuron samples from deceased patients will let researchers determine how well their converted motor neurons match natural human motor neurons. (smanewstoday.com)
- UCLA researchers have discovered the inner workings of a gene network that regulates the development of spinal motor neurons in the growing chicken and mouse embryo. (ucla.edu)
- However, researchers didn't know why or how motor neurons develop this way. (ucla.edu)
- The researchers confirmed Olig2's role in promoting motor neuron formation by increasing or blocking the function of Olig2 in the spinal cords of developing mouse and chicken embryos, as well as during motor neuron formation in mouse embryonic stem cells. (ucla.edu)
- Obtaining and protecting neural progenitor cells, as well as purified populations of motor neurons from our PCs, is a significant step for the company. (vb.is)
- Motor neuron progenitor markers (Olig2, Nkx6.1 and Pax6) and motor neuron markers (Isl1, Isl2 and Hb9) were expressed. (ac.ir)
- A method of differentiating embryonic stem cells into ventral spinal progenitor cells is disclosed. (justia.com)
- This highly improved ventral spinal progenitor and motor neuron induction over our previous method is due to continued presence of SHH in a suspension culture that promotes proliferation of the Olig2-expressing progenitors. (justia.com)
- Wang and other colleagues previously discovered a molecule (called compound C) that converts stem cells into "neural progenitor cells," an early stage in the cells' development into neurons. (blogspot.com)
- In the new study, Qu discovered that adding the signalling molecules at Day 3 worked much better: The neural progenitor cells quickly and efficiently differentiated into motor neurons. (blogspot.com)
- This indicates that Day 3 represents a previously unrecognized neural progenitor cell stage, Wang said. (blogspot.com)
- Our data thus illustrate that these lncRNAs in the Dlk1-Dio3 locus, particularly Meg3 , play a critical role in maintaining postmitotic MN cell fate by repressing progenitor genes and they shape MN subtype identity by regulating Hox genes. (yccbio.net)
- Indeed, mouse ES cells have been found to differentiate in vitro to many clinically relevant cell types, including hematopoietic cells (Wiles, M. V. and Keller, G., Development 111:259-267, 1991), cardiomyocytes (Klug, M. G., et al. (google.es)
- Its forced expression in vitro has been shown to suppress apoptosis in nonneuronal mammalian cells ( 15 ). (pnas.org)
- In this study, we report the development of low-density co-culture conditions that encourage the formation of active neuromuscular synapses between stem cell-derived MNs and muscle cells in vitro. (pubmedcentralcanada.ca)
- Through these studies we demonstrate the first evidence that astrocytes expressing mutant FUS are toxic to wild-type motor neurons in vitro. (jefferson.edu)
- In summary, I have multidisciplinary approaches, from in vitro stem cells to in vivo mouse models, to study motor neuron development and degeneration. (labroots.com)
- The in vitro characterization of fluorescence-activated cell sorting-purified cells is consistent with cells of the MN or OL lineages, depending on the stages at which they are collected. (elsevier.com)
- Moreover, hPSC-derived motor neurons were able to form neuromuscular junctions with human myotubes in vitro and induced acetylcholine receptor (AChR) clustering, as detected by Alexa 555-conjugated α-Bungarotoxin (α-BTX), suggesting that these hPSC-derived motor neurons formed functional contacts with skeletal muscles. (biomedcentral.com)
- Our ready-to-use hiPSC-derived motor neuron progenitors, developed from both healthy and patient donors, can be cultured in vitro to provide a physiologically relevant alternative to conventional animal models. (axolbio.com)
- For each step, we provide our top tips on best in vitro culture practices (based on our own research insights) to enable you to harness the enormous potential of our hiPSC-derived motor neuron progenitors as a research and drug screening tool. (axolbio.com)
- Embryonic stem (ES) cells are a unique system to model mammalian embryonic development in an accessible in vitro setting. (nyu.edu)
- We demonstrate this by recapitulating key aspects of neural tube patterning and spinal cord development in differentiating ES cells, leading to efficient production of spinal motor neurons in vitro. (nyu.edu)
- Additionally options for in-vitro testing of drug to drug candidate toxicities with well characterized alpha motor neurons for enhanced efficiency of drug discovery and improve the predictability of drug efficacy and toxicity screens. (vitrobiopharma.com)
- Maintained in α-Motor Neuron Maintaining Medium (Vitro Biopharma, Cat. (vitrobiopharma.com)
- The motor neuron-like cell line NSC-34 has become a widely used in vitro model for motor neuron biology and pathology. (elsevier.com)
- Here we report that in-vitro-expanded human fetal forebrain-derived NSCs can generate cholinergic neurons with spinal motor neuron properties when treated with bFGF within a specific time window. (utmb.edu)
- But in direct programming we do not have that: we replace the gene transcription network of the cell with a completely new one at once, without the progression through intermediate stages. (medicalnewstoday.com)
- This gene is also deleted in 3% of normal carriers ( 11 , 17 ), arguing that it is not essential for motor neuron survival or that its function can be compensated by other genes, in particular the truncated centromeric copy. (pnas.org)
- LIM homeodomain codes regulate the development of many cell types, though it is poorly understood how these factors control gene expression in a cell-specific manner. (nih.gov)
- To carry out these studies we coupled gene-profiling, quantitative protein assays, microscopy, functional assays, and pharmacological manipulations in cultures of relevant cell-types from rodents and humans, and in an acute mouse model. (jefferson.edu)
- They targeted the PAX6/ARX gene and found that selective methylation rescued the motor neurons from dysfunction. (nyscf.org)
- Survival of motor neuron gene downregulation by RNAi: towards a cell culture model of spinal muscular atrophy. (ox.ac.uk)
- SMA is caused by mutations in the survival of motor neuron gene (SMN). (ox.ac.uk)
- Left: Olig2 expression (blue) results in reduced Hes gene expression (red) and increased motor neuron formation (green) in the developing mouse spinal cord. (ucla.edu)
- Scientists used a cocktail of transcription factors involving gene expression and modifications to the chromatin which forms chromosomes within cells. (globalstemcells.com)
- The embryonic method differs from direct programming which completely replaces the gene transcription network with a new one within the cell. (globalstemcells.com)
- For this purpose, the stem cells simultaneously modify the blueprints for hundreds of proteins encoded in the gene transcripts. (stem-cells-news.com)
- Using the CRISPR/Cas9 gene editing, they have also managed to modify the cells so that they can hide from the immune system to prevent rejection. (stem-cells-news.com)
- Currently we examine how differentiating cells integrate patterning signals and translate them into lasting changes in chromatin architecture and in patterns of gene expression. (nyu.edu)
- We established a tetracycline-regulated gene expression system in this cell line by stably transfecting pTet-Off, which codifies for the tetracycline transactivator, the regulatory protein tTA. (elsevier.com)
- The monoclonal cell lines (NSC-34-tTA) were evaluated for the presence of functional tTA after transient transfection with pBI-EGFP, analyzing the expression of the reporter gene enhanced green fluorescent protein. (elsevier.com)
- The best-regulated cell line (NSC-34-tTA40) had a 66.4-fold induction for the reporter gene fluorescence in comparison to NSC-34. (elsevier.com)
- This novel cell line, NSC-34 tTA40, that permits tetracycline-regulated gene expression may prove useful to unravel the mechanisms of motor neuron degeneration. (elsevier.com)
- The converted motor neurons showed a similar genetic profile - in terms of gene activation and how they work - to mouse motor neurons. (smanewstoday.com)
- In addition, the book details gene and cell therapies that have been developed and their translation to clinical trials. (elsevier.com)
- This gave the team snapshots of global gene activity in about 200 cells that were in the process of becoming motor neurons. (ucla.edu)
- To analyze these data, the team developed custom computer software to reconstruct how gene activity changes as motor neurons form. (ucla.edu)
- The analysis suggested that a protein produced by a gene called Olig2, which is only expressed in the neural progenitors that create motor neurons, promotes motor neuron formation by interfering with the activity of several members of another gene family-the Hes genes. (ucla.edu)
- Glial cell-derived neurotrophic factor (GDNF) is a protein that, in humans, is encoded by the GDNF gene. (wikipedia.org)
Types of neurons5
- Our research revealed how small RNA molecules can work with other cell signals called transcription factors to generate specific types of neurons, in this case motor neurons. (medicalxpress.com)
- Human skin cells (above) sampled from a healthy adult and then converted into different types of neurons have the potential to be a valuable research tool. (wustl.edu)
- The research also answers a long-standing question about why motor neurons, the nerve cells of the spinal cord that control muscle movement, form much faster than other types of neurons. (ucla.edu)
- It has been known for a long time that different types of neurons form at differing speeds during development, with motor neurons forming faster than the other types of nerve cells that populate the spinal cord. (ucla.edu)
- GDNF is a small protein that potently promotes the survival of many types of neurons. (wikipedia.org)
- The approach described here may serve as a useful experimental paradigm for analyzing cell autonomy of genes found to influence structure and function of Drosophila motor neurons. (springer.com)
- In this study, we established a simple, rapid, and reproducible method for efficiently deriving motor neurons from hPSCs without the transduction of any exogenous genes. (biomedcentral.com)
- The research team used the latest molecular techniques to assess how the activity of genes change as neurons form. (ucla.edu)
- This was achieved using an approach called single cell transcriptional profiling, which allows the activity of all genes in individual cells to be measured simultaneously. (ucla.edu)
- The Hes genes are known to prevent the development of neurons. (ucla.edu)
- Glial cells under a microscope. (medicalxpress.com)
- Interestingly, the negative influence of glial cells seems to prevent motor neurons from fulfilling their normal roles, even before the motor neurons show signs of dying. (medicalxpress.com)
- Instead of developing into motor neurons like they usually would, most cells instead differentiated into a type of glial cell called a floor plate cell. (nyscf.org)
- They found that, compared to muscle cells that weren't activated, those that were produced significantly more leukemia inhibitory factor (LIF) and glial cell line-derived neurotrophic factor (GDNF), two molecular factors involved in motor neuron growth. (alsnewstoday.com)
- Astrocytes are star-shaped glial cells that have also been observed to turn into neurons by virtue of the stem cell characteristic pluripotency. (wikipedia.org)
- The body's neurons, plus the glial cells that give them structural and metabolic support, together constitute the nervous system. (wikipedia.org)
- Glial cell line-derived neurotrophic factor has been shown to interact with GFRA2 and GDNF family receptor alpha 1. (wikipedia.org)
Convert skin cells into motor neurons1
- This is the first demonstration that human iPS-derived cells are able to generate electrically active motor neurons. (ca.gov)
- The ability of ES cells to generate any cell type found in our organism can be harnessed to study how cellular diversity is established during development. (nyu.edu)
- These circuits are defined by their ability to generate rhythmic motor patterns in the absence of sensory feedback or other rhythmic inputs ( Marder and Bucher, 2007 ). (biologists.org)
- However, mechanisms by which programming factors transform cell fate are unknown, preventing more rational selection of factors to generate desirable cell types. (elsevier.com)
- All neurons are electrically excitable, due to maintenance of voltage gradients across their membranes by means of metabolically driven ion pumps, which combine with ion channels embedded in the membrane to generate intracellular-versus-extracellular concentration differences of ions such as sodium, potassium, chloride, and calcium. (wikipedia.org)
- Mutant C9orf72 human iPSC‐derived astrocytes cause non‐cell autonomous motor neuron pathophysiology, Glia (2019). (medicalxpress.com)
- 2017). Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS, Cell Reports 19 (9), 1739-1749. (nc3rs.org.uk)
- Further we demonstrate that this toxicity is mediated by activation of astrocytic NF-κB transcription factors, elevated production of the pro-inflammatory cytokine TNFα by astrocytes, and TNFα induced changes to motor neuron AMPA receptors that sensitize motor neurons to excitotoxic cell-death. (jefferson.edu)
- In particular, dysfunctional astrocytes and overactivated microglia in ALS may limit the survival of motor neurons generated from cell replacement therapy. (stemcellrevolution.com)
- Our results suggest that the neurons derived from patient-specific iPSCs can be used in drug screening including HDAC6 inhibitors targeting peripheral neuropathy. (hindawi.com)
- Patient-specific iPSCs may also provide the premises for autologous cell replacement therapies without related risks of immune rejection. (biomedcentral.com)
- Here, we review the most recent reported methods by which hESCs or iPSCs can be differentiated toward functional motor neurons with an overview on the potential clinical applications. (biomedcentral.com)
- The study was published in the journal PLOS Biology by co-senior author Bennett Novitch , member of the Eli and Edythe Broad Center of Regenerative Medicine and Stem Cell Research at UCLA and collaborators from the Francis Crick Institute in London, U.K. It has important implications for facilitating the production of motor neurons from stem cells in the lab. (ucla.edu)
- Functional and transcriptomic analysis showed that the cells have key aspects of ALS pathology including disrupted motor neuron synapse formation and mislocalisation of nuclear proteins leading to motor neuron death. (nc3rs.org.uk)
- iPS cell derived motor neurons are used in my lab to perform deep sequencing from healthy and ALS motor neurons and to functionally characterize non-coding RNA pathology in motor neuron. (labroots.com)
- Cumulatively, these cellular stresses result in synaptic pathology and cell death in VCP-mutant MNs. (crick.ac.uk)
- We additionally identify a cell-autonomous VCP-mutant AC survival phenotype, which is not attributable to the same molecular pathology occurring in VCP-mutant MNs. (crick.ac.uk)
- However, the synaptic activity of stem cell-derived MNs remains less well defined. (pubmedcentralcanada.ca)
- Together, these findings demonstrate that stem cell-derived MNs innervate muscle cells in a functionally relevant manner. (pubmedcentralcanada.ca)
- Despite these successes, relatively little attention has been placed on direct measurements of the communication between stem cell-derived MNs and muscle cells. (pubmedcentralcanada.ca)
- Stem cell derived neurons and oligodendrocytes have shown potential as therapeutics for replacement of damaged neurons and remyelination of demyelinated axons in the central nervous system (CNS). (omicsonline.org)
- In this paper, we examined the hypothesis that stem cell derived oligodendrocytes can myelinate axons of stem cell derived motor neurons in a microfluidic platform, which mimics the isolated in vivo environment. (omicsonline.org)
- Furthermore, developing a model system with stem cell-derived spinal cord motor neurons (MN) is attractive for designing control systems for soft-tissue robotics. (illinois.edu)
- However, transplanted stem cell-derived motor neurons may not survive when exposed to the harsh microenvironment in the spinal cord of ALS. (stemcellrevolution.com)
- By profiling cell types at each stage of embryonic stem cell-derived motor neurons (ESC~MNs) that recapitulate spinal cord development, we uncovered that lncRNAs expressed from the Dlk1-Dio3 locus are predominantly and gradually enriched in rostral motor neurons (MNs). (yccbio.net)
- California Stem Cell, Inc. (CSC) and Families of Spinal Muscular Atrophy (FSMA) announced today that the FDA has granted orphan drug designation to MOTORGRAFT™, a stem cell-derived motor neuron product, for the treatment of Spinal Muscular Atrophy (SMA). (news-medical.net)
- Following transplantation into the rodent central nervous system (CNS), ES cell-derived neural precursors have been shown to integrate into the host tissue and, in some cases, yield functional improvement (McDonald, J. W., et al. (google.es)
- The technique makes it possible to study motor neurons of the human central nervous system in the lab. (medicalxpress.com)
- To fully restore function, these new cells must also properly integrate into the network of cells that make up the nervous system. (eurostemcell.org)
- MND comprises a group of conditions that affect cells within the nervous system, ultimately leading to the degeneration of cells that form motor pathways - the motor neurons. (eurostemcell.org)
- There are four main types of MND, which variably affect central-nervous-system (CNS) and peripheral-nervous-system (PNS) motor pathways (otherwise known as 'upper' and 'lower' motor systems respectively). (eurostemcell.org)
- Trauma to this part of the nervous system is associated with paralysis and impairments of bladder and bowel functions, in part due to loss of motor neurons. (ca.gov)
- We further combine immunohistochemical and electrophysiological analyses with use of a collection of enhancers that tightly restrict AP1 transgene expression within the nervous system to show that AP1 induction or inhibition in, but not outside of, motor neurons is necessary and sufficient for its modulation of NMJ size and strength. (springer.com)
- Motor neurons are cells located in specific areas of the central nervous system, such as brain cortex (upper motor neurons), brain stem, and spinal cord (lower motor neurons), which maintain control over voluntary actions. (biomedcentral.com)
- thus, motor neurons are traditionally considered the last relay from the central nervous system to muscles. (biologists.org)
- Motor neurons are nerve cells of the central nervous system that transmit signals from the spinal cord to muscles to help them contract. (smanewstoday.com)
- After much trial and error, scientists have coaxed human embryonic stem cells into becoming spinal motor neurons, the critical nervous system pathways that relay messages from the brain to the rest of the body. (scienceagogo.com)
- In vertebrates, the majority of neurons belong to the central nervous system, but some reside in peripheral ganglia, and many sensory neurons are situated in sensory organs such as the retina and cochlea. (wikipedia.org)
- Somatic stem cells - also called adult stem cells - are undifferentiated cells that can be found throughout the human body in a tissue or organ. (medicalnewstoday.com)
- E ) Representative image of an established human iPSC colony, with well-defined borders and compact cells. (nih.gov)
- VAPB in human cells. (nih.gov)
- A clinical application of human ES cells would require the generation of highly purified donor cells for specific tissues and organs. (google.es)
- Following methods shown to be effective at generating motor neurons from human embryonic stem cells (hESCs), we found that once specified to a neural lineage, human iPS cells could be differentiated to form motor neurons with a similar efficiency as hESCs. (ca.gov)
- Human iPS-derived cells appeared to follow a normal developmental progression associated with motor neuron formation and possessed prototypical electrophysiological properties. (ca.gov)
- Scientists have discovered a new way to convert human skin cells directly into motor neurons (above). (medicalxpress.com)
- Human motor neurons are difficult to study since they can't be taken from living patients. (medicalxpress.com)
- Past work by Yoo and his colleagues-then at Stanford University-showed that exposure to two short snippets of RNA turned human skin cells into neurons. (medicalxpress.com)
- Using a rapid and efficient protocol to differentiate mouse embryonic stem cells (ESC) to SpMNs and CrMNs, we now report that ESC-derived CrMNs accumulate less human superoxide dismutase 1 (hSOD1) and insoluble p62 than SpMNs over time. (ssrn.com)
- The project aims to demonstrate that transplantation of cortical motor neurons derived from human iPS will contribute to functional and anatomical repair after SCI through implementing an active relay graft and improve synaptic plasticity and circuitry. (wingsforlife.com)
- Human ES cell‐derived neural rosettes reveal a functionally distinct early neural stem cell stage. (currentprotocols.com)
- Scientists at the University of St Andrews and the University of Edinburgh have identified a specific type of cell in the human body which can cause motor neurons to fail using stem cell technology. (medicalxpress.com)
- In this project, we grow human embryonic stem cells to develop into immature motor neurons, and these cells are transplanted into the spinal cord of rats with lumbosacral nerve root injuries and repair. (ca.gov)
- Our studies on bladder wall thickness and on the thickness of the bladder lining showed no differences between control groups and treatment groups that had received human motor neurons as cell transplants into the spinal cord. (ca.gov)
- We conclude that the human cell-based therapies did not inflict any adverse effects on the bladder wall, which represents the end-organ in our injury and repair model. (ca.gov)
- The PC program expands on the Company's currently marketed C3A human liver cell-based toxicology offerings. (vb.is)
- Needed in the art is a simple, yet efficient, strategy for the isolation of transplantable neural and motor neuron precursors from differentiating human ES cell cultures. (justia.com)
- These motor neurons might be a great research tool, creating a model for investigating the cellular and molecular interactions underlying early human brain development and pathologies during neurodegeneration. (biomedcentral.com)
- The TDP-43 in the motor neurons of the human spinal cord has also been found to be a mRNA-binding protein of low molecular weight microfilament [ 4 ]. (ijbs.com)
- There are hundreds of specific types of motor neurons that are required in a figure as complex as the human body. (sophiascure.org)
- There is a tremendous amount of work left to be done before we can create all of the different motor neurons in the human body and the exact placement of each of these motor neurons along the spine. (sophiascure.org)
- They did this by exposing skin cells in a lab to certain molecular signals usually found at high levels in the human brain. (healthscienceandtechnology.com)
- "We would argue that whatever happens in the human body is going to be quite efficient, quite rapid," said University of Illinois cell and developmental biology professor Fei Wang , who led the study with visiting scholar Qiuhao Qu and materials science and engineering professor Jianjun Cheng . (blogspot.com)
- So it's unlikely that those methods recreate human motor neuron development. (blogspot.com)
- The new approach will allow scientists to induce mature human motor neuron development in cell culture, and to identify the factors that are vital to that process, Wang said. (blogspot.com)
- Scientists have struggled to grow human motor neurons in the lab for research purposes, which is one reason this work is so notable. (smanewstoday.com)
- How well their genetic profile compares to human motor neurons is still a question, because these cells are very difficult to obtain from living adults. (smanewstoday.com)
- The financing will enable the company to support the advancement of its therapeutic products to human clinical trials, as well as the generation and distribution of its tools to aid in stem cell research and drug discovery. (news-medical.net)
- However progress to a treatment is hampered by the problem of delivering the drug to brain cells through the blood-brain barrier in human. (wikipedia.org)
- In motor neurons, however, Isl1 is available to compete for binding to NLI, displacing Lhx3 to a high-affinity binding site on the C-terminal region of Isl1 and thereby transforming Lhx3 from an interneuron-promoting factor to a motor neuron-promoting factor. (nih.gov)
- Three transcription factors, Ngn2, Isl1 and Lhx3, were sufficient to program rapidly and efficiently spinal motor neuron identity when expressed in differentiating mouse embryonic stem cells. (elsevier.com)
- Chromatin immunoprecipitation-sequencing analysis of Isl1, Lhx3 and Phox2a binding sites revealed that the two cell fates were programmed by the recruitment of Isl1-Lhx3 and Isl1-Phox2a complexes to distinct genomic locations characterized by a unique grammar of homeodomain binding motifs. (elsevier.com)
- We further discovered that purmorphamine, a small molecule that activates the SHH pathway, could substitute SHH for the generation of ventral spinal progenitors and motor neurons. (justia.com)
- Transitioning to hiPSC-derived motor neuron progenitors takes just a few simple steps, which we outline below. (axolbio.com)
- Axol's hiPSC-derived motor neuron progenitors arrive at the bench ready to use, so you don't need to waste any time getting started. (axolbio.com)
- Culturing hiPSC-derived motor neuron progenitors is typically a straightforward process. (axolbio.com)
- Expect the hiPSC-Derived motor neuron progenitors to grow as aggregated clumps, similarly to that shown in Figure 1. (axolbio.com)
- It is normal to see the hiPSC-derived motor neuron progenitors growing as aggregated clumps. (axolbio.com)
- Similar to mouse ES cells, they can be expanded to large numbers while maintaining their potential to differentiate into various somatic cell types of all three germ layers (Thomson, J. A., et al. (google.es)
- The University of Washington describes a motor unit as consisting of a somatic motor or efferent neuron and all the muscle fibers or cells it stimulates or innervates. (reference.com)
Spinal motor neuron1
Cortical motor neurons1
Functional motor neurons1
- The motor neurons that innervate muscle fibres are essential for motor activity. (eurekalert.org)
- In thoracic segments this Lim3-negative population became MNs in the lateral division of the medial motor column that innervate intercostal muscles, whereas in the cervical spinal cord this population disappeared by E5 because of cell death. (nii.ac.jp)
- The motor neurons can 'reach' through small channels in the device to innervate (stimulate) the muscle cells, forming a modeled neuromuscular junction. (alsnewstoday.com)
- Peripheral motor neurons relay signals from the brain to the muscle through a synapse called the neuromuscular junction (NMJ). (eurostemcell.org)
- Astrocytic TNFα additionally signals onto endothelial cells to increase drug-efflux properties which drive pharmaco-resistance. (jefferson.edu)
- Motor neurons send signals to muscle fibers, telling them when to contract. (alsnewstoday.com)
- The space between a motor neuron (nerve cell) and a muscle cell, where these signals are sent, is called the neuromuscular junction (NMJ). (alsnewstoday.com)
- Motor behaviors depend on neural signals in the brain. (biologists.org)
- A neuron, also known as a neurone and nerve cell, is an electrically excitable cell that receives, processes, and transmits information through electrical and chemical signals. (wikipedia.org)
- Most neurons receive signals via the dendrites and send out signals down the axon. (wikipedia.org)
- We examined the TDP-43 expression and distribution in the different anatomic regions, segments and neural cells in the adult spinal cord at the different stages of the SOD1 wild-type and G93A transgenic model by the fluorescent immunohistochemical technology. (ijbs.com)
- They are the motor neurones of the spine, in which SOD1 mutations lead to mis-shapen, dysfunctional units of the enzyme superoxide dismutase 1 (SOD1) can 'decouple' the normal processes of energy exchange and use in the mitochondria of these cells. (evolving-science.com)
- We urgently need large, well-designed clinical trials to establish whether or not cell-based therapies have a clear clinical benefit in ALS/MND. (cochrane.org)
- These functioning neuron and oligodendrocyte units may be a very useful tool to study stem cell replacement therapies for nerve injuries where nerve reconstruction would be beneficial. (omicsonline.org)