DiseasesChemicals and DrugsPsychiatry and PsychologyPhenomena and Processes
Cerebellar DiseasesPrion DiseasesGait AtaxiaCerebellar AtaxiaPrionsPrPSc ProteinsPrPC ProteinsPsychomotor PerformanceCreutzfeldt-Jakob SyndromeScrapieEndopeptidase KInsomnia, Fatal FamilialGerstmann-Straussler-Scheinker DiseaseWasting Disease, ChronicEncephalopathy, Bovine SpongiformKuru
AtaxiaExpressed prion proteinDegenerationProteinDisordersAlzheimer'sBovine Spongiform EncSymptomsCellularCreutzfeldt-Jakob DIsoformParkinson's DiseaseAnimal prionNeuronsCerebralReplicationSporadicProteinsDysfunctionScrapie in sheepAbnormalNeurodegenerationSheepMultiple sclerosisNeurological diseaseDystoniaClinicalPathologyAbstractSubtypesAtaxiasParkinsonianAlzheimerAtrophyIncubationBrain homogenateNystagmusHumanGeneticSpongiform encephalopathyInducesChronic wastingFatalAccumulation
Ataxia5
  • It results in devastating outcome of mental deterioration, hypokinesia, myoclonus and cerebellar signs of nystagmus and ataxia. (pmrjabstracts.org)
  • Upon disease progression, BSE-affected macaques displayed ataxia and tremors as first signs of neuronal defects. (biomedcentral.com)
  • Cerebellar compromise leads to ataxia and other extrapyramidal signs such as hypokinesia, bradykinesia, and dystonia. (picmonic.com)
  • Autoantibodies to glutamic acid decarboxylase in three patients with cerebellar ataxia, late-onset insulin-dependent diabetes mellitus, and polyendocrine autoimmunity. (jamanetwork.com)
  • One third of people, however, may present with cerebellar symptoms such as dysarthria, falls and truncal ataxia. (vic.gov.au)
Expressed prion protein1
  • The replication of prions involves the recruitment of the normally expressed prion protein, which has mainly an alpha-helical structure, into a disease-specific conformation that is rich in beta-sheet. (medscape.com)
Degeneration15
  • Spongiform encephalopathy consists of prion induced spongiform degeneration of the cerebral cortex and cerebellum. (picmonic.com)
  • Best's Disease - a form of two-way central (macular) retinal pigment abiotrophy leading to the degeneration of photoreceptors and macular significant vision loss. (medicalformat.com)
  • Approximately 55 percent were felt to have a single progressive neurodegenerative etiology, predominantly Alzheimer disease (AD), frontotemporal dementia (FTD), dementia with Lewy bodies (DLB), dementia related to Parkinson disease (PD), and corticobasal degeneration (including corticobasal syndrome and many other phenotypes) [ 2 ]. (medilib.ir)
  • Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of prions. (usda.gov)
  • Greenlee JE, Brashear HR. Antibodies to cerebellar Purkinje cells in patients with paraneoplastic cerebellar degeneration and ovarian carcinoma. (jamanetwork.com)
  • Paraneoplastic cerebellar degeneration, I: a clinical analysis of 55 anti-Yo antibody-positive patients. (jamanetwork.com)
  • Tumor-specific killer cells in paraneoplastic cerebellar degeneration. (jamanetwork.com)
  • Cerebellar degeneration with Hodgkin disease: an immunological study. (jamanetwork.com)
  • Reduction of P/Q-type calcium channels in the postmortem cerebellum of paraneoplastic cerebellar degeneration with Lambert-Eaton myasthenic syndrome. (jamanetwork.com)
  • However, even in the more common and usually slowly progressive dementias such as Alzheimer's disease, frontotemporal lobar degeneration, dementia with Lewy bodies and other degenerative dementias, as well as vascular dementia, establishment and progression of the disease is occasionally surprisingly accelerated, leading to a clinical presentation of RPD. (touchneurology.com)
  • Séverine BOILLEE's team investigates mechanisms of motor neuron (MN) degeneration in Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's disease) resulting from pathological interactions between MNs and microglia/macrophages to find therapeutically promising pathways to slow disease progression. (sorbonne-universite.fr)
  • The primary cause of degeneration is still under debate- whether cell loss in MSA is secondary to disruptions in the oligo-myelin-axon complex [ 29 , 64 ], or if MSA is a primary neuron disease, with the secondary formation of GCIs following pathological accumulation of α-syn that is neuronal in origin [ 55 ]. (biomedcentral.com)
  • There are many other diseases and conditions that cause NDCs, including vascular dementia, frontotemporal degeneration, Lewy body disease, and prion disease. (lumenlearning.com)
  • The presentation of degenerative disease in focal areas of the cerebral cortex is the hallmark of the family of diseases referred to as frontotemporal dementia (also termed frontotemporal lobar degeneration). (medscape.com)
  • In CNS and peripheral nervous system diseases associated with axonal injury or degeneration, the concentration of neurofilament light chain (NfL) has been found to increase in cerebrospinal fluid (CSF) and blood. (bmj.com)
Protein32
  • The infectious agent in the prion disease is composed mainly or entirely of an abnormal conformation of a host-encoded glycoprotein called the prion protein. (medscape.com)
  • These amyloid plaques are immunoreactive with antibodies to the prion protein and do not immunoreact with antibodies to other amyloidogenic proteins, such as the amyloid-beta (which is deposited in Alzheimer disease). (medscape.com)
  • Highly divergent hypotheses have been put forward regarding the makeup of the prions, including that they consist of nucleic acid only or protein only, are lacking both protein and nucleic acid, or are a polysaccharide. (medscape.com)
  • BSE is thought to be due to an infection by a misfolded protein, known as a prion. (wikipedia.org)
  • BSE prions are misfolded forms of the particular brain protein called prion protein. (wikipedia.org)
  • Although the role of abnormal prion protein (PrP) conformation in generating infectious brain diseases (transmissible spongiform encephalopathy) has been recognized, the function of PrP in the normal brain remains mostly unknown. (amrita.edu)
  • The prion gene and its encoded prion protein have long been known to play a central role in prion diseases. (usda.gov)
  • Recently, a second gene and its protein (shadoo) have also been implicated in prion disease. (usda.gov)
  • Background: The cellular prion protein PrP**C is encoded by the Prnp gene. (usda.gov)
  • This protein is expressed in the central nervous system (CNS) and serves as a precursor to the misfolded PrP**S**c isoform in prion diseases. (usda.gov)
  • Sho protein levels are reduced in prion infections in rodents. (usda.gov)
  • The causative agent is or is closely related to a pathogenic isoform of the cellular prion protein (PrP c ). (biomedcentral.com)
  • Cali I , Puoti G , Smucny J, Curtiss PM, Cracco L, Kitamoto T, Occhipinti R, Cohen ML , Appleby BS, Gambetti P . Co-existence of PrP types 1 and 2 in sporadic Creutzfeldt-Jakob disease of the VV subgroup: phenotypic and prion protein characteristics. (academictree.org)
  • Camacho MV, Telling G , Kong Q , Gambetti P , Notari S . Role of prion protein glycosylation in replication of human prions by protein misfolding cyclic amplification. (academictree.org)
  • Cracco L, Xiao X, Nemani SK, Lavrich J, Cali I , Ghetti B, Notari S , Surewicz WK , Gambetti P . Gerstmann-Sträussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments. (academictree.org)
  • Prion disease is caused by a misfolded prion protein that has a predominantly beta-sheet structure. (picmonic.com)
  • This misfolded protein is resistant to the actions of proteases and can induce conformational changes in normal cellular prion proteins. (picmonic.com)
  • Kuru is among the fatal neurodegenerative prion protein (PrP) diseases in humans. (medscape.com)
  • The prion is a naturally occurring protein found in the CNS and elsewhere. (medscape.com)
  • According to the protein-only hypothesis, an abnormal PrP isoform is the principal, and possibly sole, constituent of the transmissible agent or prion. (medscape.com)
  • The central feature of this protein was a posttranslational conversion of the host-encoded cellular prion protein (PrPC) to an abnormal isoform, termed PrPSc, that consists of ''small proteinaceous infectious particles that resist inactivation by procedures which modify nucleic acids," ie, radiation, heat, or enzymatic degradation. (medscape.com)
  • PrP C , the cellular isoform of the prion protein, serves to transduce the neurotoxic effects of PrP Sc , the infectious isoform, but how this occurs is mysterious. (elifesciences.org)
  • Prion diseases are a group of degenerative illnesses of the brain caused when a molecule called the prion protein (PrP for short) adopts the wrong shape. (elifesciences.org)
  • These diseases are caused by refolding of the cellular prion protein (PrP C ) into an infectious isoform (PrP Sc ) that catalytically templates its abnormal conformation onto additional molecules of PrP C ( Prusiner, 1998 ). (elifesciences.org)
  • The infectious agent is a unique abnormal prion protein, designated as PrP. (vic.gov.au)
  • The disease is strongly linked to the consumption of cattle products infected with the prion protein that causes bovine spongiform encephalopathy (BSE) or 'mad cow' disease. (vic.gov.au)
  • The human cellular prion protein (PrP C ) is a glycosylphosphatidylinositol (GPI) anchored membrane glycoprotein with two N-glycosylation sites at residues 181 and 197. (mdpi.com)
  • This resembles the β-amyloid precursor protein (APP) in Alzheimer disease (AD), which can be physiologically processed by α-, β-, and γ-secretases. (mdpi.com)
  • This protein is already known in connection with Alzheimer's disease and recent studies prove that CBD can lower levels of this protein and decrease its degenerative activity. (moya-cbd.co.uk)
  • It's now known that MSA is a prion disease - that is, it's a neurological disease caused by exposure to a new type of protein. (neurology-clinics.com)
  • Is indeed the prion protein an Harlequin servant of 'many' masters? (unipd.it)
  • Milisav I, Šuput D, Ribarič S. Unfolded protein response and macroautophagy in Alzheimer's, Parkinson's and prion diseases. (sci-rep.com)
Disorders22
  • Part of the Oxford Textbooks in Clinical Neurology series, the Oxford Textbook of Neuroimaging provides an overview of the established and latest neuroimaging methodologies, and illustrates their application to the main diseases of the brain and the spinal cord including movement disorders, headache and stroke. (pickapdf.com)
  • I discuss issues pertaining to the practice of neuropathology -- including nervous system tumors, neuroanatomy, neurodegenerative disease, muscle and nerve disorders, ophthalmologic pathology, neuro trivia, neuropathology gossip, job listings and anything else that might be of interest to a blue-collar neuropathologist. (blogspot.com)
  • Overview of Movement and Cerebellar Disorders Voluntary movement requires complex interaction of the corticospinal (pyramidal) tracts, basal ganglia, and cerebellum (the center for motor coordination) to ensure smooth, purposeful movement. (msdmanuals.com)
  • FND is a clinical entity with "rule-in" signs that are designed to distinguish it from recognized or standard neurological disease, according to the Diagnostic and Statistical Manual of Mental Disorders . (trialbyerror.org)
  • The article in the Journal of Neurology is not the first report related to FNDs and prion disorders. (trialbyerror.org)
  • Treatment - symptomatic and pathogenetic therapy of the underlying disease, correction of neurological and psychiatric disorders. (medicalformat.com)
  • Von Willebrand disease - congenital disorders of hemostasis, which manifests itself quantitative and qualitative shortage of plasma von Willebrand factor and bleeding disorders. (medicalformat.com)
  • Similar mechanisms may also contribute to other life-threatening brain disorders, including Alzheimer's disease and Parkinson's disease. (elifesciences.org)
  • A better understanding of the role of PrP in prion diseases may help to reveal new treatments for these and other degenerative brain disorders. (elifesciences.org)
  • Prion diseases, or transmissible spongiform encephalopathies, comprise a group of fatal neurodegenerative disorders in humans and animals for which there are no effective treatments or cures. (elifesciences.org)
  • Various conditions involving the central nervous system (CNS) can emerge as RPD, including Creutzfeld-Jakob disease (CJD) and other spongiform encephalopathies, vascular disorders, autoimmune and paraneoplastic encephalopathies, subacute infections, metabolic and toxic disorders and systemic diseases (see Table 1 ). (touchneurology.com)
  • However, it is important to point out that even neurodegenerative disorders such as Alzheimer's disease (AD), dementia with Lewy bodies and frontotemporal dementia present in rare cases as a subacute dementia instead of a slowly progressive deterioration of higher functions. (touchneurology.com)
  • Prion diseases are rare, invariably lethal and rapidly progressive neurodegenerative disorders that affect humans and other species of mammals [ 1 ]. (biomedcentral.com)
  • This work also identified two other loci associated with an increased risk of sCJD, in STX6 ( rs3747957) and GAL3ST1 (rs2267161) genes, indicating intracellular trafficking and sphingolipid metabolism as probable triggering mechanisms and corroborating the likely shared molecular dysregulation with other prion-like disorders [ 10 ]. (biomedcentral.com)
  • As we have seen, neurocognitive disorders may be caused by several medical conditions, such as Alzheimer's disease, Parkinson's disease, and Huntington's disease because they affect or deteriorate brain functions. (lumenlearning.com)
  • Most of these disorders stemmed from untreated infectious diseases secondary to AIDS (acquired immunodeficiency syndrome) like toxoplasmosis and encephalitis. (lumenlearning.com)
  • Its levels increase in cerebrospinal fluid (CSF) and blood proportionally to the degree of axonal damage in a variety of neurological disorders, including inflammatory, neurodegenerative, traumatic and cerebrovascular diseases. (bmj.com)
  • This applies to central nervous system (CNS) disorders of all causes, including inflammatory, neurodegenerative, traumatic and vascular diseases. (bmj.com)
  • Since it is feasible to measure NfL concentration in the blood, it may be a promising biomarker for monitoring the disease course in CNS disorders and, ideally, for evaluating patients' response to treatments. (bmj.com)
  • Alok Sahay, MD, has been a neurologist at the Gardner Center for Parkinson's Disease and Movement Disorders in the University of Cincinnati Neuroscience Institute since 2001. (uchealth.com)
  • Dr. Sahay is currently a professor of neurology at the University of Cincinnati College of Medicine, and a physician in the UC Department of Neurology and the Cincinnati Veterans Administration Medical Center focusing on movement disorders and Parkinson's disease. (uchealth.com)
  • This group includes lysosomal storage disorders, various mitochondrial diseases, other neurometabolic disorders, and several other miscellaneous disorders. (medscape.com)
Alzheimer's5
  • In post-mortem examinations carried out on the brains of people who died as a result of diseases such as Alzheimer's, Parkinson's, and Huntington's, the researchers found an imbalance in the endocannabinoid system. (moya-cbd.co.uk)
  • A microglial activity state biomarker panel differentiates FTD-granulin and Alzheimer's disease patients from controls. (cdc.gov)
  • and tau pathologies and their relationships to cognitive dysfunction in Alzheimer's disease. (cdc.gov)
  • Psychopharmacological Medication Use in Frontotemporal Dementia at the Time of Diagnosis: Comparison with Alzheimer's Disease. (cdc.gov)
  • Klotho Gene Expression Is Decreased in Peripheral Blood Mononuclear Cells in Patients with Alzheimer's Disease and Frontotemporal Dementia. (cdc.gov)
Bovine Spongiform Enc5
  • [ 1 ] Included are Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker (GSS) in humans, bovine spongiform encephalopathy (BSE, or "mad cow disease") in cattle, chronic wasting disease (CWD) in mule deer and elk, and scrapie in sheep. (medscape.com)
  • For related information, see Medscape Reference article Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy . (medscape.com)
  • Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is an incurable and invariably fatal neurodegenerative disease of cattle. (wikipedia.org)
  • Transmissible spongiform encephalopathies (TSE) comprise a group of neurodegenerative diseases including bovine spongiform encephalopathy (BSE) in cattle, Scrapie in sheep, and Creutzfeldt-Jakob disease (CJD) in humans [ 1 ]. (biomedcentral.com)
  • Bovine spongiform encephalopathy (BSE) first came to the attention of the scientific community in November 1986 with the appearance of a newly recognized form of neurological disease in cattle in the United Kingdom. (who.int)
Symptoms8
  • These diseases all have long incubation periods but are typically rapidly progressive once clinical symptoms begin. (medscape.com)
  • Creutzfeldt-Jakob disease is a neurodegenerative disorder under the umbrella of prion diseases with long incubation periods and progress inexorably once symptoms appear. (pmrjabstracts.org)
  • Despite the well-known symptoms of Wilson disease, sexual dysfunction is also a frequent issue in Wilson disease patients, necessitating further attention. (bvsalud.org)
  • These changes led to symptoms in animal models of the diseases. (elifesciences.org)
  • Creutzfeldt-Jakob disease (CJD) is characterized by a broad phenotypic spectrum regarding symptoms, progression, and molecular features. (biomedcentral.com)
  • It is characterized by a wide phenotypic spectrum regarding first symptoms, disease progression, and histo-molecular features. (biomedcentral.com)
  • Moreover, the student must have a deep knowledge of the most common neurological diseases- being able to connect symptoms and signs with diseases. (unicampania.it)
  • Approximately 15% of patients follow a primary progressive or progressive relapsing course from disease onset, usually characterized by symptoms of progressive myelopathy (gait instability, spasticity, bladder symptoms) and cognitive impairment. (medscape.com)
Cellular1
  • The processing of APP has been extensively studied, while the identity of the cellular proteases involved in the proteolysis of PrP C and their possible role in prion biology has remained limited and controversial. (mdpi.com)
Creutzfeldt-Jakob D13
  • Spread to humans is believed to result in variant Creutzfeldt-Jakob disease (vCJD). (wikipedia.org)
  • Neurological workup completed revealed positive lumbar puncture for 14-3-3 proteins and real-time quakin-induced conversion (Rt-QuIC) study making diagnosis of sporadic Creutzfeldt-Jakob disease (CJD) overwhelmingly positive. (pmrjabstracts.org)
  • The aim of our study was to analyze the differential expression of miRNAs in the brains of BSE-infected cynomolgus macaques as a model for Creutzfeldt-Jakob disease (CJD). (biomedcentral.com)
  • To investigate whether prion-induced neurodegeneration is linked to deregulation of miRNA in the brain of affected individuals, we analyzed differential miRNA expression in brains of BSE-infected non-human primates ( Macaca fascicularis ) as a model for Creutzfeldt-Jakob disease in humans. (biomedcentral.com)
  • Creutzfeldt-Jakob Disease (CJD) Creutzfeldt-Jakob disease (CJD) is the most common human prion disease. (msdmanuals.com)
  • Phenotypic diversity of genetic Creutzfeldt-Jakob disease: a histo-molecular-based classification. (academictree.org)
  • Cali I , Espinosa JC, Nemani SK, Marin-Moreno A, Camacho MV, Aslam R, Kitamoto T, Appleby BS, Torres JM, Gambetti P . Two distinct conformers of PrP type 1 of sporadic Creutzfeldt-Jakob disease with codon 129VV genotype faithfully propagate in vivo. (academictree.org)
  • Identification of novel risk loci and causal insights for sporadic Creutzfeldt-Jakob disease: a genome-wide association study. (academictree.org)
  • Creutzfeldt-Jakob disease is a prion disorder that leads to dementia and death, usually within months. (trialbyerror.org)
  • Creutzfeldt-Jakob disease (CJD) is the most common cause of spongiform encephalopathy. (picmonic.com)
  • Others include Creutzfeldt-Jakob disease (CJD), Gerstmann-Straüssler-Scheinker (GSS) disease, fatal familial insomnia (FFI), and variant CJD (vCJD). (medscape.com)
  • Creutzfeldt-Jakob disease (CJD) must be notified by medical practitioners and pathology services in writing within 5 days of diagnosis. (vic.gov.au)
  • Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common and best studied human prion disease. (biomedcentral.com)
Isoform2
  • Prion diseases are associated with an accumulation of a disease-related isoform of host-encoded PrP through a posttranslational process involving conformational change and aggregation. (medscape.com)
  • A possible mechanism for prion propagation involves the largely alpha-helical isoform (PrPC) refolding into a beta-sheet isoform (beta-PrP). (medscape.com)
Parkinson's Disease3
  • The results highlight a different functional impairment, with VV2 associated with higher impairment of the pathways related to dopamine secretion, regulation of calcium release and GABA signaling, showing some similarities with Parkinson's disease both on a genomic and a transcriptomic level. (biomedcentral.com)
  • The results add to the understanding of the molecular features associated with sporadic CJD and its most common subtypes, revealing strain-specific genetic signatures and functional similarities between VV2 and Parkinson's disease and providing preliminary evidence of RNA editing modifications in human sCJD. (biomedcentral.com)
  • Olga CORTI & Jean-Christophe CORVOL's team proposes a multidimensional clinical, genetic/genomic and cell biology-based program aimed at deciphering and integrating the molecular heterogeneity and biological complexity of Parkinson's Disease (PD) towards translation to clinical research. (sorbonne-universite.fr)
Animal prion1
  • Although cross-species transmission of prion diseases seems to be limited by an apparent species barrier, the epidemic of BSE in the United Kingdom, which began in 1986, and its transmission to humans indicated that animal prion diseases could pose a significant public health risk. (medscape.com)
Neurons2
  • Recently, the CNS glycoprotein Shadoo (Sho) has been shown to resemble PrP**C, both in a central hydrophobic domain and in activity in a toxicity assay performed in cerebellar neurons. (usda.gov)
  • Diseases of neuronal cell disability have few things in common and are a disorder in biochemical processes in neurons: problems with neural conduction, oxidative stress, activation of microglia [MG] cells (microglia are the local macrophages in the brain), and poor function of transferring vital proteins in the brain and cells of the central nervous system [CNS]. (moya-cbd.co.uk)
Cerebral1
  • Cerebral vascular diseases are associated with brain injuries, for example, autoimmune deficiencies are associated with damage caused to the body by its immune system. (moya-cbd.co.uk)
Replication1
  • First, there is a potential issue with the kinetics of prion replication. (cureffi.org)
Sporadic2
  • Human prion diseases can be divided etiologically into inherited, sporadic, and acquired forms. (medscape.com)
  • Recent research aimed mainly at a better understanding of the genetic risk factors and modifiers associated with the onset and phenotypic expression of the sporadic disease: Genome-Wide Association Studies (GWAS) of large sCJD cohorts confirmed the significant association with PRNP codon 129, the strongest genetic risk factor [ 10 ]. (biomedcentral.com)
Proteins4
  • The prion induces normally-folded proteins to take on the misfolded phenotype in an exponential cascade. (wikipedia.org)
  • CJD is caused by the misfolded prion proteins which have a high affinity for brain tissue and induce further conformational changes of physiological prion proteins. (picmonic.com)
  • Normally, prion proteins are encoded by the PRNP gene and exist in a mostly alpha-helix configuration. (picmonic.com)
  • Nevertheless, there is a strong correlation between the neurotoxicity caused by prion proteins and the blockade of their normal proteolysis. (mdpi.com)
Dysfunction5
  • It is called scrapie because of the tendency of affected animals to rub against the fences of their pens in order to stay upright, reflecting their cerebellar dysfunction. (medscape.com)
  • In this study, we aimed to investigate current status of sexual dysfunction in Wilson disease. (bvsalud.org)
  • Our study identified sexual dysfunction as a prevalent issue in Wilson disease patients, with an overall prevalence of 49.0 %, of which 33.9 % in males and 63.7 % in females, both higher than the prevalence of sexual dysfunction in the normal Chinese population. (bvsalud.org)
  • Misfolded prions are resistant to proteases, and their accumulation causes intracellular dysfunction as well as extracellular aggregation (plaques). (picmonic.com)
  • Multiple system atrophy (MSA) is a fatal neurodegenerative disorder that presents clinically with varying combinations of autonomic, parkinsonian, cerebellar, and pyramidal dysfunction [ 15 ]. (biomedcentral.com)
Scrapie in sheep1
  • The prototype prion disease is scrapie in sheep, and, whereas, Prnp exhibits common missense polymorphisms for V136A, R154H and Q171R in ovine populations, genetic variation in mouse Prnp is limited. (usda.gov)
Abnormal2
  • Though the normal activity of PrP is not certain, abnormal PrP can affect the healthy PrP on the surface of brain cells and lead to disease. (elifesciences.org)
  • PrP acts on normal prions, causing them to change into the abnormal infectious form in a cascade-like manner. (vic.gov.au)
Neurodegeneration2
  • The molecular mechanism underlying prion-induced neurodegeneration is still poorly understood. (biomedcentral.com)
  • Neurodegenerative diseases of the nervous system are debilitative and incurable medical conditions that lead to neurodegeneration and/or death of nerve cells. (moya-cbd.co.uk)
Sheep6
  • The first of these diseases to be described was scrapie, a disease of sheep recognized for over 250 years. (medscape.com)
  • The transmission of this disease was demonstrated first in 1943 when a population of Scottish sheep was accidentally inoculated against a common virus using a formalin extract of lymphoid tissue from an animal with scrapie. (medscape.com)
  • Cattle are believed to have been infected by being fed meat-and-bone meal (MBM) that contained either the remains of cattle who spontaneously developed the disease or scrapie-infected sheep products. (wikipedia.org)
  • Variant type (vCJD) is due to the consumption of meat from cows or sheep with prions in their muscle tissue. (picmonic.com)
  • Scrapie is a disease mainly affecting sheep between two and five years of age. (who.int)
  • It is a prion disease that can harm sheep. (articleted.com)
Multiple sclerosis3
  • The remaining 45 percent of patients had the following etiologies: vascular-related cognitive change, alcohol-related cognitive change, Huntington disease (HD), cognitive impairment resulting from multiple sclerosis (MS), prion diseases, dementia related to Down syndrome (predominantly AD), and unknown/unclassified. (medilib.ir)
  • Multiple sclerosis is a common, chronic demyelinating neurological disease primarily affecting young adults, with a prevalence of ~0.1% in the Caucasian population (Miller and Leary, 2007). (medscape.com)
  • Diagnostic criteria and classification of multiple sclerosis subtypes have evolved in recent decades, and, although successive versions have differed in emphasis, all have required dissemination of disease in space (requiring involvement of multiple areas of the CNS) and in time (requiring ongoing disease activity over time). (medscape.com)
Neurological disease2
  • The counter-argument is that that misdiagnosing someone as having a known neurological disease when they actually have FND can also cause harm. (trialbyerror.org)
  • Oral examination.The oral exam covers all the aspects of the theory of neurological disease. (unicampania.it)
Dystonia1
  • Wilson disease is a rare neurogenetic disorder that receives significant attention due to its manifestations, such as jaundice, cirrhosis, tremor, dystonia, and others. (bvsalud.org)
Clinical8
  • This problem leads to the thought: if only we had the ability to do phenotypic screening in human cells infected with human prions, maybe we'd have a small molecule in clinical trials by now. (cureffi.org)
  • The pathological and clinical characteristics of kuru that distinguish it from other PrP diseases in humans are also discussed. (medscape.com)
  • The few published case series of RPD have shown that the relative frequency of underlying diseases depends mainly on the clinical setting. (touchneurology.com)
  • Diagnosis is by the clinical presentation, disease progression and exclusion of other causes. (vic.gov.au)
  • The main objective in this research area is to develop markers and therapy for neurological and psychiatric diseases, from identification on simple models in laboratories to clinical trials with patients at ICM's Clinical Investigation Center and throughout the Institute. (sorbonne-universite.fr)
  • Evidence that both CSF and blood NfL may serve as diagnostic, prognostic and monitoring biomarkers in neurological diseases is progressively increasing, and NfL is one of the most promising biomarkers to be used in clinical and research setting in the next future. (bmj.com)
  • Another application for biomarkers in neurological diseases could be to identify or rule out the presence of neurodegenerative processes, which would be useful for subsequent clinical management. (bmj.com)
  • We then review its potential diagnostic and prognostic value in a variety of CNS diseases, as well as its usefulness in monitoring response to treatment, and we discuss how NfL could be applied in clinical practice. (bmj.com)
Pathology1
  • Disease Gallervordena-Spatz - a hereditary neurodegenerative pathology caused by the deposition of iron in the basal ganglia of the brain. (medicalformat.com)
Abstract1
  • Palatulan E, Beckley A. Rehabilitation of a Patient with Prion's Disease [abstract]. (pmrjabstracts.org)
Subtypes2
  • This work investigates the molecular basis of the phenotypic heterogeneity of prion diseases through a multi-omics analysis of the two most common sCJD subtypes: MM1 and VV2. (biomedcentral.com)
  • DNA target sequencing identified multiple potential genetic contributors to the disease onset and phenotype, both in terms of coding, damaging-predicted variants, and enriched groups of SNPs in the whole cohort and the two subtypes. (biomedcentral.com)
Ataxias1
  • Alexandra DURR & Giovanni STEVANIN's team focus on two groups of neurogenetic diseases, spinocerebellar degenerations - SCD (spastic paraplegias and cerebellar ataxias) and frontotemporal lobar degenerations - FTLD. (sorbonne-universite.fr)
Parkinsonian1
Alzheimer2
  • Nevertheless, the most common causes of early-onset dementia are the same in younger and older adults: Alzheimer disease (AD), vascular dementia, and frontotemporal dementia (FTD). (medilib.ir)
  • While this definition recognizes multiple different cognitive domains, a decline in memory is one of the earliest and most prominent features of Alzheimer disease (AD) dementia and most other forms of dementia. (medilib.ir)
Atrophy1
  • His case study "On the relationship between aphasia and senile atrophy of the brain" still serves as a frame of reference for apparently focal brain syndromes in diffuse or generalized degenerative diseases of the brain. (medscape.com)
Incubation1
  • however, the disease has persisted into the present century because of an incubation period that may exceed 50 years. (medscape.com)
Brain homogenate1
  • Which is: dump prion-infected brain homogenate on cells expressing human PrP, passage them enough times to dilute the original prion material to a negligible quantity, possibly subclone to try to identify and expand individual clones that are infected, and Western blot after proteinase K digestion to see if you've got new prions generated by the cells. (cureffi.org)
Nystagmus1
  • Other cerebellar manifestations include nystagmus. (picmonic.com)
Human14
  • The conclusions, findings, and opinions expressed by authors contributing to this journal do not necessarily reflect the official position of the U.S. Department of Health and Human Services, the Public Health Service, the Centers for Disease Control and Prevention, or the authors' affiliated institutions. (cdc.gov)
  • Efforts to prevent the disease in the UK include not allowing any animal older than 30 months to enter either the human food or animal feed supply. (wikipedia.org)
  • Shadoo expression has been linked to mouse and human prion diseases, thus, investigating shadoo will shed light onto prion diseases and their potential prevention and cure. (usda.gov)
  • His studies in the multiple aspects of developmental biology and early nervous system development are key to gaining a greater insight into the pathogenesis of human diseases, including brain malformations, mental retardation, epilepsy and autism. (blogspot.com)
  • Therefore, we hypothesized that miRNAs are also regulated in response to human prion disease. (biomedcentral.com)
  • With respect to the reported regulation of this miRNA in Scrapie-infected mice, we propose that upregulation of hsa-miR-342-3p may be a general phenomenon in late stage prion disease and might be used as a novel marker for animal and human TSEs. (biomedcentral.com)
  • Thus we hypothesized that miRNA regulation may also play a role in human prion diseases. (biomedcentral.com)
  • Above: some human IMR32 neuroblastoma cells that are, like all human immortalized cell lines, not propagating prions. (cureffi.org)
  • If anything, the data would hint that there's something unique about human prions that makes infection im possible, because at this point people have succeeded with virtually every oft-studied species except human. (cureffi.org)
  • [ 16 ] A common coding polymorphism at codon 129 of the PrP gene ( PRNP ), where either methionine (M) or valine (V) may be encoded, is a strong susceptibility factor for human prion diseases. (medscape.com)
  • These diseases include the human form of mad cow disease, and are often fatal with no effective treatments or cures. (elifesciences.org)
  • This group of re-emerging human and animal diseases has recently attracted much attention, as well as concern, both in the scientific world and among the general public. (who.int)
  • Critically, demyelination was observed in the white matter tracts of the corpus callosum and striatum of Olig001-α-syn but not Olig001-GFP injected animals, similar to the human disease. (biomedcentral.com)
  • This course aims at introducing students to the concept of animal health, care, disease recognition, prevention and biosecurity, as well as the importance and role of veterinary sciences in the human societies. (uaeu.ac.ae)
Genetic3
  • This raises the possibility that, like the prion gene, genetic variation in shadoo may influence prion disease. (usda.gov)
  • Gerstmann-Sträussler-Scheinker syndrome is a genetic prion disorder. (trialbyerror.org)
  • Search Directory of Genetic and Rare Diseases Information Center (GARD) provides the public with access to current, reliable, and easy-to-understand information about rare or genetic diseases in English or Spanish. (defeatmsa.org.nz)
Spongiform encephalopathy1
  • Prions in cows cause spongiform encephalopathy in cows, famously known as mad cow disease. (picmonic.com)
Induces1
  • When physiological prion changes its composition from alpha helix to predominantly beta sheet, it induces similar changes in other prions. (picmonic.com)
Chronic wasting1
  • In the United States, chronic wasting disease occurs in mule deer and elk. (who.int)
Fatal2
Accumulation1
  • A characteristic feature of Wilson's disease - the accumulation of copper in various organs and tissues, mainly in the liver, and the basal ganglia. (medicalformat.com)