CHRONIC WASTING DISEASE OF ELK AND DEER AND CREUTZFELDT-JAKOB DISEASE: COMPARATIVE ANALYSIS OF THE SCRAPIE PRION PROTEIN*

Chronic wasting disease (CWD), a transmissible prion disease that affects elk and deer, poses new challenges to animal and human public health. Although the transmission of CWD to humans has not been proven, it remains a possibility. If this were to occur, it is important to know whether the “acquired ” human prion disease would show a phenotype including the scrapie prion protein (PrPSc) features that differ from those associated with human sporadic prion disease. In this study, we have compared the pathological profiles and PrPSc characteristics in brains of CWD-affected elk and deer with that in subjects with sporadic Creutzfeldt