PRNP and human prion disease: Such distinct disease-associated PrP assembly states from Gerstmann-Sträussler-Scheinker (GSS) patients with the P102L PrP mutation transmit different phenotypes to experimental reporter mice resulting in either a lethal transmissible spongiform encephalopathy (associated with transmission of classical PrPSc) or a clinically silent PrP amyloidosis (associated with the transmission of the PrP conformer generating an approximately 8 kDa, protease-resistant PrP fragment) [33–35].