BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein
作者： Emmanuel A Asante , Jacqueline M Linehan , Melanie Desbruslais , Susan Joiner , Ian Gowland
DOI: 10.1093/EMBOJ/CDF653
关键词: Creutzfeldt-Jakob Syndrome 、 Transmissible mink encephalopathy 、 Genotype 、 Transgene 、 Bovine spongiform encephalopathy 、 Phenotype 、 Virology 、 Biology 、 Scrapie 、 Genetics 、 PRNP
摘要: Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent these diseases being caused by same prion strain. Surprisingly, however, BSE transmission mice, addition producing a vCJD-like phenotype, can also result distinct is indistinguishable from sporadic PrPSc type 2. These data suggest more than one BSE-derived strain might infect humans; it therefore possible some patients have arising exposure.
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