Tg(Prnp-HTT*82Q)52Caro

This transgene expresses the N-terminal 586 amino acids of human huntingtin (Htt) cDNA with a polyglutamine repeat length of 82, under the control of the mouse prion protein promoter. Amino acid 586 is the putative cleavage site of Htt by caspase 6 and so this transgene contains the caspase 6 fragment that would result from cleavage by caspase 6. This represents an intermediate-sized fragment. The mouse prion protein promoter drives transgene expression in the neurons of the central nervous system. Western blots show wide distribution of the protein in all brain regions tested. Immunoblots show that the N586-82Q fragment is cleaved into smaller fragments over time. Two lines were created, 52 and 91. Line 52 is a high-expressing line. (J:179364)