De novo prions

Abstract

Prions are responsible for a heterogeneous group of fatal neurodegenerative diseases. They occur in three forms - sporadic, genetic, or acquired - and involve non-covalent post-translational modifications of the cellular prion protein (PrPC). Prions (PrPSc) are characterized by their infectious properties and intrinsic ability to act as a template, converting the normal, physiological PrPC into the pathological form, PrP Sc. The 'protein-only' hypothesis, postulated by Stanley B Prusiner, implies that the generation of de novo prions is possible. Exciting recent work, in vivo and in vitro, has further strengthened this postulate. © 2010 Faculty of 1000 Ltd.