About The Medical Condition Prion Protein Disease

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Find the definition of the medical term Prion Protein Disease. Prion Protein Disease defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.

Prion Protein Disease

Prion Protein Disease Medical Condition Defined & Explained
Prion Protein Disease Medical Condition

What's The Definition Of The Medical Condition Prion Protein Disease?

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. Diseases in this category include CREUTZFELDT-JAKOB SYNDROME; GERSTMANN-STRAUSSLER-SCHEINKER SYNDROME; KURU; SCRAPIE; fatal familial insomnia; bovine spongiform encephalopathy (see ENCEPHALOPATHY, BOVINE SPONGIFORM); transmissible mink encephalopathy; and chronic wasting disease of mule deer and elk. Pathologic features include a spongiform encephalopathy without evidence of inflammation. The literature occasionally refers to these as unconventional SLOW VIRUS DISEASES. (From Proc Natl Acad Sci USA 1998 Nov 10;95(23):13363-83)

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