PRNP and human prion disease: In the brain PrPC may adopt an amyloidogenic, partially protease-resistant conformation enriched in β-sheet secondary structures known as prion or PrPSc [12], which is related to a class of human and animal neurodegenerative diseases denoted as transmissible spongiform encephalopathies (TSE) including scrapie in sheep and goat, chronic wasting disease (CWD) in cervids and bovine spongiform encephalopathy (BSE) in cattle [13].