Tg(Prnp-FTL*)4Ruvi

The mouse promoter was used to drive brain and heart specific expression of the human cDNA with TC insertions at the cDNA positions 498 and 499, respectively. This insertion leads to a frame-shift that alters the last 9 amino acids of the C-terminus and adds 16 additional amino acids. Four lines were created (lines 1, 2, 3, and 4) with 40 to 50, 5 to 10, 1 to 5, and 1 to 5 copies of the transgene, respectively. Line 4 was used for subsequent studies. (J:131053)