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Since its identification as a distinct form of human prion disease, it has been demonstrated that vCJD is related to bovine spongiform encephalopathy (BSE),1 thus providing evidence for transmission of the disease from cattle to humans. Despite widespread beef consumption, however, the number of cases of vCJD has been low and moreover, there is no history of unusual exposure to beef or its products among affected persons,1 These findings may arise from a combination of factors, including the existence of environmental factors that may affect susceptibility, the long incubation period for vCJD, uneven exposure to infected beef, and variations in individual genetic susceptibility to the transmission process. Of the known genetic factors, it has been established that polymorphisms of codon 129 of the prion protein gene confer individual susceptibility to vCJD.1 However, this polymorphism is common in the normal population, suggesting …