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Human prion diseases have inherited, sporadic, and acquired etiologies. The appearance of the novel acquired prion disease, variant Creutzfeldt-Jakob disease (vCJD), and the demonstration that it is caused by the same prion strain as that causing bovine spongiform encephalopathy, has led to fears of a major human epidemic. The etiology of classical (sporadic) CJD, which has a worldwide incidence,...
The human prion protein gene (PRNP) encodes a 33–35 kDa cell surface protein that is highly expressed in the central nervous system and is vital to the pathogenesis of prion diseases. We have characterised the promoter region of PRNP as a first step towards defining the mechanisms regulating its expression. Sequence analysis of a 2.7 kb genomic DNA fragment containing exon I and the 5′-flanking region...
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