We initially tested a single SNP, rs12249854(A/T), located in a HECTD2 intron, and showed that the minor allele (A) was significantly over-represented in vCJD (n = 117, 8.1%) compared to controls (n = 601, 3.9%), P = 0.0049, (OR 2.11, 95% CI 1.19–3.77, trend test 1 d.f.), and between sporadic CJD (n = 452, 6.3%) and controls, P = 0.012, (OR 1.65, 95% CI 1.11–2.46, trend test 1 d.f.). This evidence concerns the gene HECTD2 and variant Creutzfeldt-Jakob disease.