摘要：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, PrPSc. 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.
