Human Molecular Genetics, Vol 6, 1699-1705, Copyright © 1997 by Oxford University Press
J Collinge
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.
REVIEWS
Human prion diseases and bovine spongiform encephalopathy (BSE)
Prion Disease Group, Neurogenetics Unit, Imperial College School of Medicine at St. Mary's, London, UK. j.collinge@ic.ac.uk
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