The accumulation of misfolded PrP characterizes prionoses, such as Creutzfeldt-Jakob disease, Kuru, Gerstmann–Sträussler–Scheinker Syndrome, and fatal familial insomnia in humans, bovine spongiform encephalopathy in cattle, scrapie in sheep, and chronic wasting disease in deer and elk (84, 85, 86). This evidence concerns the gene PRNP and scrapie.