Definition of Prion disease

Prion disease: A disease due to a prion, a proteinaceous infectious particle that lacks nucleic acids. Prions are composed largely, if not entirely, of an altered form (an abnormal isoform) of a normal cellular protein.

The known prion diseases of humans and other mammals are:

• Bovine spongiform encephalopathy (BSE) -- also called mad cow disease; the natural hosts are cattle; the prion involved is the BSE prion.
• Creutzfeldt-Jakob disease (CJD) -- the natural hosts are humans; the prion involved is the CJD prion.
• Gerstmann-Straussler-Scheinker syndrome (GSS) -- the natural hosts are humans; the prion involved is the GSS prion.
• Fatal familial insomnia (FFI) -- the natural hosts are humans; the prion involved is the FFI prion.
• Kuru -- the natural hosts are humans; the prion involved is the kuru prion.
• Scrapie -- the natural hosts of this disease are sheep and goats; the prion involved is the scrapie prion.
• Transmissible mink encephalopathy (TME) -- the natural hosts of this disease are mink; the prion involved is the TME prion.
• Chronic wasting disease (CWD) -- the natural hosts of this disease are mule deer, white-tailed deer, black-tailed deer, and elk; the prion involved is the CWD prion.
• Feline spongiform encephalopathy (FSE) -- the natural hosts are cats; the prion involved is the FSE prion.
• Exotic ungulate encephalopathy (EUE) -- the natural hosts are the nyala and greater kudu; the prion involved is the EUE prion.

See also: Prusiner, Stanley B..