Prion diseases are caused by the accumulation of protease-resistant misfolded form (PrPSc) rich in the beta-sheet structure of cellular prion protein (PrPC), which results in astrogliosis and neuronal loss and vacuolation, eventually leading to brain dysfunction and inevitable death (Gu et al., 2002; Minikel et al., 2020; Sevillano et al., 2020). The gene discussed is PRNP; the disease is prion disease.