| prion | The word, for proteinaceous infectious agent, was coined in 1982 by neurologist Stanley Prusiner as part of a hypothesis regarding ailments bearing aetiologic resemblance to those caused by slow viruses (for instance, kuru). The hypothesis has been borne out by investigation. Prions are now believed responsible for several transmissible neurodegenerative diseases Origin: proteinaceous infectious particle (05 Mar 2000) |
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| prion diseases | Transmissible and genetic neurodegenerative diseases of humans and animals caused by prions. The diseases are usually characterised by vacuolation in the gray matter and result in ataxia, motor disturbances, dementia, and progression to a fatal outcome. They include creutzfeldt-jakob syndrome, gerstmann-straussler syndrome, kuru, scrapie, fatal familial insomnia, bovine spongiform encephalopathy (encephalopathy, bovine spongiform), transmissible mink encephalopathy, and chronic wasting disease of mule deer and elk. The literature has sometimes referred to these as unconventional slow virus diseases. (12 Dec 1998) |
| prion protein | Small, infectious proteinaceous particle, of non-nucleic acid composition because of its resistance to nucleases; the causative agent, either on a sporadic, genetic, or infectious basis, of six neurodegenerative diseases in animals, and four in humans; the latter include the spongiform encephalopathies of kuru, Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia. The gene encoding for the PrP is found on chromosome 20. Synonym: prion. (05 Mar 2000) |
| prions | See: PrP, Gerstmann Straussler Scheinker syndrome. Suggested as the causative agents of several infectious diseases such as scrapie (in sheep), kuru and Creutzfeld Jakob disease in man. Prions (proteinaceous infective particles) apparently contain no nucleic acid. The 27 kD protein of scrapie is related to a normal cell protein and may possibly cause its over production. (18 Nov 1997) |