Prion diseases are a groupof neurodegenerative disorders thatare characterized by the accumulation of the abnormal isoform (PrPSc) of prion protein (PrP) in the central nervous system.1 Prion diseases have three etiologies, sporadic,inherited, and acquired, depending on how the causative PrPSc propagation is initiated in the body. Here, PRNP is linked to prion disease.