PRNP and Gerstmann-Straussler-Scheinker syndrome: It is well established that similar PrPSc fragments also characterize the so-called inherited prion protein amyloidoses, including Gerstmann-Sträussler-Scheinker disease (GSS), and the cerebral and systemic amyloidosis that are linked to stop-codon truncating PRNP mutations [33].