First all-human mouse model of inherited prion disease

Human prion diseases include Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler-Scheinker disease (GSS). A new study reports a significant advance in the development of mouse models of human prion diseases. The study demonstrates spontaneous formation of disease-relevant, transmissible prion protein assemblies in mice bearing only human forms of the prion protein.