Article
Human prion protein with valine 129 prevents expression of variant CJD phenotype.
Science (New York, N.Y.) - 3 Dec 2004
Wadsworth Jonathan D F, Asante Emmanuel A, Desbruslais Melanie, Linehan Jacqueline M, Joiner Susan, Gowland Ian, Welch Julie, Stone Lisa, Lloyd Sarah E, Hill Andrew F, Brandner Sebastian, Collinge John
Abstract excerpt
Variant Creutzfeldt-Jakob disease (vCJD) is a unique and highly distinctive clinicopathological and molecular phenotype of human prion disease associated with infection with bovine spongiform encephalopathy (BSE)-like prions. Here, we found that generation of this phenotype in transgenic mice required expression of human prion protein (PrP) with methionine 129. Expression of human PrP with valine 129 resulted in...
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