Prion diseases are a varied group of rare, fatal neurodegenerative diseases. They may be inherited or acquired.
The common pathological finding is the deposition of an insoluble form of a host-encoded protein, prion protein.
Public interest was been stimulated by the link between a British epidemic of bovine spongiform encephalopathy, a bovine prion disease, and a variant of Creutzfeldt-Jakob disease.
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