Tg(ATXN3*)84.2Cce

A 250 kb YAC containing the intact human locus with 15 CAG trinucleotide repeats in exon 10 was modified by homologous recombination to expand the number of CAG repeats to 84. Two copies of the transgene inserted into this line with the number of CAG repeats for both copies confirmed by sequencing (a second founder line, 84.1, had insertion of 2 transgenes with variant triplets and altered repeat numbers). Transgene expression was detected in the cerebellum at endogenous levels and was also detected in the cerebral cortex, heart, and skeletal muscle. (J:76495)