Inherited Prion Diseases (IPDs), such as familial Creutzfeldt-Jakob Disease (fCJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), and Fatal Familial Insomnia (FFI), constitute a subset of prion disorders caused by mutations in PRNP. Here, PRNP is linked to Gerstmann-Straussler-Scheinker syndrome.