Tg(Prnp-PRNP)361Jmto

Mice with this transgene express the Val129 variant of human prion protein under control of the mouse prion protein regulatory sequences. The transgene was generated by substitution of the open reading frame (ORF) of the human prion protein gene for the ORF in the third exon of the orthologous mouse gene in the expression vector MoPrP.Xho. This vector contains the mouse genomic sequence including exon 1, intron 1, and exon 2 joined directly to exon 3, in which the ORF has been replaced by an Xho1 restriction endonuclease site, thus providing the promoter, 5'-untranslated region (5'-UTR), and 3'-UTR from the mouse locus. Expression of the transgene-encoded protein in brains of homozygous transgenic mice is ~4-fold that in human brain, as determined by densitometric immunoblot analysis of serially diluted brain homogenates using the 3F4 monoclonal antibody, which recognizes the 109MKHM112 epitope of the human prion protein. (J:225676, J:235784)