Dictionary of Human Evolution and Biology

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Transmissible Spongiform Encephalopathies

Any of several fatal degenerative diseases, allegedly caused by a prion, which create spongiform abscesses in brain tissue, and accompanied by progressive muscular and cognitive impairment. The human diseases in this group include kuru, new variant Creutzfeldt-Jakob disease (nvCJD), fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker syndrome (GSSS). The prion protein gene (PRNP) on chromosome 20 is responsible. Analogous animal diseases include scrapie, transmissible mink encephalopathy, chronic wasting of deer and elk (wapiti), bovine spongiform encephalopathy (mad cow disease), feline spongiform encephalopathy, and exotic ungulate encephalopathy. These appear to be limited to regions where certain varieties of sheep reside, and to countries where these same sheep varieties (or, subsequently, cattle) have been exported. The potential for similar prion diseases in humans exists wherever prion-infected animals are consumed, e.g. deer and elk.

See the protein-only hypothesis and prion protein.

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