Journal Article

Human Prion Diseases and Bovine Spongiform Encephalopathy (BSE)

John Collinge

in Human Molecular Genetics

Volume 6, issue 10, pages 1699-1705
Published in print September 1997 | ISSN: 0964-6906
Published online September 1997 | e-ISSN: 1460-2083 | DOI: http://dx.doi.org/10.1093/hmg/6.10.1699
Human Prion Diseases and Bovine Spongiform Encephalopathy (BSE)

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Prion diseases are transmissible neurodegenerative disorders which affect a range of mammalian species. In humans they can be inherited and sporadic as well as acquired by exposure to human prions. Prions appear to be composed principally of a conformational isomer of host-encoded prion protein and propagate by recruitment of cellular prion protein. Recent evidence argues that prion protein can also encode disease phenotypes by differences in its conformation and glycosylation. Such molecular analysis of prion strains suggests that new variant Creutzfeldt-Jakob disease is caused by BSE exposure. The novel biology of prion propagation may not be unique to these rare degenerative brain diseases.

Journal Article.  6679 words.  Illustrated.

Subjects: Genetics and Genomics

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