PRNP/CD230

PRNP/CD230 is the cellular prion protein PrPC, encoded by the PRNP gene, a GPI-anchored glycoprotein of 25-35 kDa abundantly present on neurons and widely expressed in the CNS and peripheral tissues. Its structure features an unstructured N-terminal region with octapeptide repeats that bind copper, and a C-terminal domain with three alpha-helices and a short beta-sheet. While its physiological roles in synaptic function, copper homeostasis and antioxidant defense remain debated, misfolding of PrPC into the scrapie form PrPSc is the pivotal event in transmissible spongiform encephalopathies such as Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Prion protein aggregates propagate by templated misfolding and resist conventional sterilization.