Prnp^tm3Lnq

The third exon was replaced with an HPRT mini gene that was then replaced with a representative sequence containing two amino acid substitutions found in the human sequence (L108M and V111M). These substitutions allow for transmission barrier against mouse scrapies and immunoreactivity to the 3F4 antibody. A third mutation (E199K) mimics the mutation associated with human Creutzfeldt Jakob disease. Protein expression levels in the brains of homozygous mice are similar to controls. (J:200974)