Prion disease, also known as transmissible spongiform encephalopathy, is characterized by the accumulation of scrapie prion proteins (PrPSc), the disease-associated isoform of the cellular prion proteins (PrPC), in the periphery and central nervous system (CNS) during disease progression [[1], [2], [3]]. The gene discussed is PRNP; the disease is human prion disease.