Article
Dissociation of pathological and molecular phenotype of variant Creutzfeldt-Jakob disease in transgenic human prion protein 129 heterozygous mice.
Proceedings of the National Academy of Sciences of the United States of America - 11 Jul 2006
Asante Emmanuel A, Linehan Jacqueline M, Gowland Ian, Joiner Susan, Fox Katie, Cooper Sharon, Osiguwa Olufumilayo, Gorry Michelle, Welch Julie, Houghton Richard, Desbruslais Melanie, Brandner Sebastian, Wadsworth Jonathan D F, Collinge John
Abstract excerpt
All neuropathologically confirmed cases of variant Creutzfeldt-Jakob disease (vCJD), characterized by abundant florid plaques and type 4 disease-related prion protein (PrP(Sc)) in the brain, have been homozygous for methionine at polymorphic residue 129 of PRNP. The distinctive neuropathological and molecular phenotype of vCJD can be faithfully recapitulated in Prnp-null transgenic mice homozygous for human PrP...
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