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Disease Prion Diseases

Name Prion Diseases
Synonyms Dementias, Transmissible | Dementia, Transmissible | Disorder, Prion-Induced | Disorders, Prion-Induced | Encephalopathies, Spongiform, Transmissible | Encephalopathies, Transmissible Spongiform | Encephalopathy, Transmissible Spongiform | Human Transmissible Spongiform Encephalopathies, Inherited | Inherited Human Transmissible Spongiform Encephalopathies | Prion-Associated Disorders | Prion Disease | Prion Induced Disorder | Prion-Induced Disorder | Prion-Induced Disorders | Prion Protein Disease | Prion Protein Diseases | Spongiform Encephalopathies, Transmissible | Spongiform Encephalopathy, Transmissible | Transmissible Dementia | Transmissible Dementias | Transmissible Spongiform Encephalopathies | Transmissible Spongiform Encephalopathy
Definition A group of genetic, infectious, or sporadic degenerative human and animal nervous system disorders associated with abnormal PRIONS. These diseases are characterized by conversion of the normal prion protein to an abnormal configuration via a post-translational process. In humans, these conditions generally feature DEMENTIA; ATAXIA; and a fatal outcome. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The older literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)
Categories Nervous system disease
MeSH® ID D017096
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Top ↑ Ancestors

1. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Central Nervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Central Nervous System Infections Has associated chemicals Has associated genes Prion Diseases Has associated chemicals Has associated genes
2. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Neurodegenerative Diseases Has associated chemicals Has associated genes Has associated exposure references Prion Diseases Has associated chemicals Has associated genes

Top ↑ Descendants

Prion Diseases Has associated chemicals Has associated genes
  Amyloidosis, Cerebral, with Spongiform Encephalopathy
View node Creutzfeldt-Jakob Syndrome Has associated chemicals Has associated genes
View node Encephalopathy, Bovine Spongiform Has associated chemicals Has associated genes
  Gerstmann-Straussler-Scheinker Disease Has associated chemicals Has associated genes
  Insomnia, Fatal Familial Has associated chemicals Has associated genes
  Kuru Has associated chemicals Has associated genes
  Scrapie Has associated chemicals Has associated genes
  Spongiform Encephalopathy with Neuropsychiatric Features Has associated chemicals Has associated genes
  Wasting Disease, Chronic