α-cleavage of the prion protein occurs in a late compartment of the secretory pathway and is independent of lipid rafts

AR Walmsley, NT Watt, DR Taylor, WSS Perera… - Molecular and Cellular …, 2009 - Elsevier
Endoproteolysis of the cellular prion protein (PrPC) modulates both the normal function of
the protein and the pathogenesis of the neurodegenerative prion diseases. PrPC undergoes
α-cleavage to generate the N-terminally truncated fragment C1. Utilizing various constructs
of PrPC expressed in human neuroblastoma cells we investigated the subcellular
compartment where α-cleavage occurs. C1 was detected at the cell surface and the
generation of C1 occurred in mutants of PrPC incapable of Cu2+-mediated endocytosis. A …