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prion

Pronunciation: pri'on

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]

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.

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