Tg(ATN1*)Q129Stsu

The transgene was engineered from BAC DRPLA18E that contains the human gene for ATN1 from a patient with dentatorubral-pallidoluysian atrophy (DRPLA) with a 76 CAG trinucleotide repeat within the gene. During maintenance of Tg(ATN1*)Drm21Stsu a mosaic mouse was isolated contained a combination of cells with 76 CAG repeats and 129 CAG repeats within the transgene. This mouse was bred to produce a line with the mutant human ATN1 with 129 CAG repeats. (J:144379)