Transmissible spongiform encephalopathies (TSEs) or prion diseases are caused by conformational transitioning of the physiological host-encoded prion protein, PrPC, into a misfolded and aggregated form, PrPSc that accumulates in the brain in the form of plaques or smaller plaque-like deposits [1, 2]. The gene discussed is PRNP; the disease is human prion disease.