PRNP and human prion disease: The latter, also known as transmissible spongiform encephalopathies, are caused by the conformational conversion of a cell surface glycoprotein, named the cellular prion protein (PrP), into an aggregated, pathogenic form (called PrP scrapie, or PrPSc) capable of propagating like an infectious agent (prion) by templating the structural conversion of its physiological counterpart7,8.