Simulations and computational analyses of prion protein conformations

Abstract

Publisher Summary Prions are transmissible pathogens that cause fatal neurodegenerative diseases of the central nervous system in humans and animals. In humans, prion diseases have been subdivided into four classes and are referred to as kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), and fatal familial insomnia (FFI). These diseases can be sporadic, inherited, or infectious (or iatrogenic) disorders. They differ from other infectious diseases, in that the pathogen is a proteinaceous particle, termed a “prion,” and the essential component of prions is the scrapie prion protein (PrP Sc ).