Prion diseases, such as bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep, and Creutzfeldt-Jakob disease (CJD), kuru and Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans, are a group of neurodegenerative disorders caused by prions, self-replicating β-sheet-rich infectious polymeric assemblies of misfolded host-encoded cellular prion protein (PrPC)1–4. Here, PRNP is linked to Creutzfeldt Jacob disease.