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Pathogenesis

HLA-DQ7 antigen and resistance to variant CJD

Abstract

Variant Creutzfeldt–Jakob disease (vCJD) in humans is caused by a bovine spongiform encephalopathy (BSE)-like prion strain, and so far about 100 of the many people exposed to BSE prions have developed the condition. Here we show that there is a significantly reduced frequency of the human leukocyte antigen (HLA) class-II type DQ7 in patients with vCJD, but not in those with classical CJD. This apparently protective genetic factor should aid differential diagnosis and may have important implications for understanding host susceptibility to infection by BSE prions, and the distinctive pathogenesis of vCJD, as well as in the identification of targets for prevention and therapy of vCJD.

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Correspondence to John Collinge.

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Jackson, G., Beck, J., Navarrete, C. et al. HLA-DQ7 antigen and resistance to variant CJD. Nature 414, 269–270 (2001). https://doi.org/10.1038/35104694

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