The prion (proteinaceous infectious only) was defined in 1982 by Stanley B. Prusiner as the infectious agent of transmissible spongiform encephalopathies (TSEs). Development and progression of the disease implies ongoing structural conversion of the highly conserved cellular prion protein (PrPC ) into a partially protease-resistant isoform (PrPSc ). This applies to
all human TSEs, such as Creutzfeldt-Jakob disease, Gerstmann-Straeussler-Scheinker syndrome, Kuru and fatal familial insomnia, and for animal TSEs as well (scrapie in sheep and goats, bovine spongiform encephalopathy [BSE], and chronic wasting disease of deer and elk).
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