Prion protein (PrP) amyloid plaques are found in postmortem brain samples from subjects diagnosed with prion-related transmissible spongiform encephalopathies (TSEs), including Creutzfeldt-Jakob disease (CJD), hereditary Gerstmann-Straussler-Scheinker syndrome, kuru, and the animal prion disease scrapie (Liberski, 1994). Here, PRNP is linked to human prion disease.