Experience of other acquired human prion diseases, kuru and iatrogenic CJD [4,5], and extensive transmission studies in transgenic mice expressing human PrP argued that further cases might occur in the other PRNP codon 129 genotypes (VV and MV) with longer incubation periods and with different disease phenotypes [1,2,6–14]. Here, PRNP is linked to Creutzfeldt Jacob disease.