Prnpsc is the major pathological Prnp conformer that present in high quantities in the brains of animals and humans with infectious neurodegenerative prion diseases, such as sheep scrapie, bovine spongiform encephalopathy, Creutzfeldt-Jacob disease, and Gerstmann-Straussler syndrome [48]. Here, PRNP is linked to Creutzfeldt Jacob disease.