PRNP and prion disease: Prion diseases (transmissible spongiform encephalopathies, TSE) are recognized with the pathogenetic mechanism of protein misfolding of the prion protein (PrP), which shifts from a physiological conformation (PrPC) into a protease-resistant, amyloidogenic isoform, named PrP “scrapie” (PrPSc)7–9.