The disease progression is accompanied by the loss of cognitive skills and neuronal dysfunction and can be of inherited sporadic or iatrogenic origin.2, 3 The central pathophysiologic event is ascribed to the conformational change of the cellular prion protein (PrPC) into scrapie prion protein (PrPSc) that then not only propagates further PrPC misfolding in neighboring cells but can also infect other organisms.4 Identification of the infective pathogen of prion diseases and its proof of transmissibility started in the 1950s. Here, PRNP is linked to prion disease.