Prion Protein Isoforms, a Convergence of Biological and Structural Investigations

Prion diseases are fatal neurodegenerative disorders afflicting humans and other mammals. Manifest as sporadic, inherited, and infectious illnesses, they seem to be the result of a conformational transition in the normal, cellular prion protein (PrPC) 1 whereby the aberrant, diseased protein isoform (PrPSc) is formed(1). The transmissible particle or prion is composed largely, if not exclusively, of PrPSc and is resistant to procedures that modify nucleic acids(2, 3). No candidate scrapie-specific polynucleotide has been identified(4).