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BSE prions propagate as either variant CJD‐like or sporadic CJD‐like prion strains in transgenic mice expressing human prion protein
Author(s) -
Asante Emmanuel A.,
Linehan Jacqueline M.,
Desbruslais Melanie,
Joiner Susan,
Gowland Ian,
Wood Andrew L.,
Welch Julie,
Hill Andrew F.,
Lloyd Sarah E.,
Wadsworth Jonathan D.F.,
Collinge John
Publication year - 2002
Publication title -
the embo journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.484
H-Index - 392
eISSN - 1460-2075
pISSN - 0261-4189
DOI - 10.1093/emboj/cdf653
Subject(s) - bovine spongiform encephalopathy , biology , prnp , phenotype , virology , genetically modified mouse , transgene , fatal familial insomnia , transmissible spongiform encephalopathy , prion protein , disease , creutzfeldt jakob syndrome , methionine , scrapie , genetics , gene , genotype , pathology , medicine , amino acid
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 methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD‐like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrP Sc type 2. These data suggest that more than one BSE‐derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.