PRNP and Creutzfeldt Jacob disease: For instance, upon inoculation with different human and animal prion strains, transgenic mice expressing the BvPrP with methionine or isoleucine at codon 109 (TgM109 or TgI109, respectively) developed prion pathology and all generated PrPres were characterized by a predominance of the di-glycosylated band, even in those challenged with human or animal prion strains with the mono-glycosylated predominant PrP band (e.g., sporadic CJD or Rocky Mountain Laboratory prion strain (RML)) (Watts et al., 2014).