Tg(Prnp*D177N*M128V)G1Rchi

The transgene construct contained a a 12 kb genomic DNA fragment of the mouse Prnp gene, including the promoter and first intron, joined to a cDNA encoding the a variant of mouse prion protein in which aspartic acid is replaced by asparagine at anino acid position 177 (D177N) and methionine by valine at position 128 (M128V), equivalent to the hereditary Creutzfeldt-Jakob disease- (CJD-) associated human D178N/V129 prion protein mutation. Hemizygous mice of transgenic line G1 carry 6 copies of the transgene; western blot analysis detects the mutant prion protein in brains of these mice at 0.15 times the endogenous PRNP level in wild-type brains. The gross pattern of mutant PRNP expression in brains of transgenic mice is similar to that of endogenous PRNP in non-transgenic mice. (J:142098)