Definition: An infectious proteinaceous particle of nonnucleic acid composition; the causative agent, either on a sporadic, genetic, or infectious basis, of neurodegenerative diseases in animals, and humans. The latter include the spongiform encephalopathies of kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker (GSS) syndrome, and fatal familial insomnia. The gene encoding prion protein (PrP) occurs on chromosome 20.
Synonym(s): prion protein
[proteinaceous infectious particle]