The present data (i) add to previous studies reporting the rare occurrence of white matter PrP amyloid plaques in patients with an otherwise classic sCJDMM1 phenotype; (ii) originally report the occurrence of white matter PrP plaque-like deposits in genetic CJDMM1 and the results of the experimental transmission of p-sCJDMM1 to bank voles, and (iii) further address the issue of the molecular basis of amyloid plaque formation in CJD by providing an extensive characterization of the physico-chemical properties of PrPSc aggregates in p-CJDMM1. Here, PRNP is linked to Creutzfeldt Jacob disease.