Prion diseases, which also include scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans, are fatal diseases produced by the aberrant misfolding of the cellular prion protein (PrPC) into an infectious isoform (PrPCWD) which accumulates in the brain causing neurodegeneration1. The gene discussed is PRNP; the disease is Creutzfeldt Jacob disease.