PRNP and prion disease: In prion diseases, PrPC is converted into PrPSc, which can work via template-mediated misfolding to further convert host PrPC protein into a variety of misfolded forms that aggregate and accumulate within the nervous tissue (Will and Ironside, 1999; Budka, 2003; Soto and Castilla, 2004; Linden et al., 2008).