A conformational transition of the cellular prion protein (PrP^C) into an aberrantly folded isoform designated scrapie prion protein (PrP^Sc) is the hallmark of a variety of neurodegenerative disorders collectively called prion diseases. They include Creutzfeldt-Jakob disease and Gerstmann-Stäussler-Scheinker syndrome in humans, scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle and chronic wasting disease (CWD) in free-ranging deer.

In contrast to the deadly properties of misfolded PrP, PrP^C seems to possess a neuroprotective activity. More-over, animal models indicated that the stress-protective activity of PrP^C and the neurotoxic effects of PrP^Sc are somehow interconnected

from The Prion Protein

Further reading:

• The Prion Protein

Labels: Bovine spongiform encephalopathy, BSE, Chronic wasting disease, Creutzfeldt-Jakob disease, Gerstmann-Staussler-Scheinker syndrome, Prion, Prion disease, PRP, PRPC, PRPSc, Scrapie