Prnp^tm2Lnq

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 (D177N) mimicks the mutation associated with human fatal familial insominia. Protein expression levels in the brains of homozygous mice are reduced compared to controls. (J:154937)