The conformational conversion of the cellular prion protein (PrPC) into its β-sheet-rich scrapie isoform (PrPSc) causes fatal prion diseases, which are also called transmissible spongiform encephalopathies (TSEs).The TSEs include scrapie, bovine spongiform encephalopathy (BSE), and human Creutzfeld-Jacob disease (CJD) [1], [2]. Here, PRNP is linked to Creutzfeldt Jacob disease.