This transgene consists of the rat prion promoter, lacZ reporter gene, and 500 ATTCT repeats of human ATXN10 (isolated from a Mexican patient with ataxia and epilepsy) inserted in the 3' UTR of lacZ. RT-PCR analysis shows that the transgene is widely expressed in the central nervous system, with highest expression in the hippocampus, and also in the heart, liver, muscle, and skin. Three founders (1,2, 3) were established, however the pound symbol (#) is used when line is not specified and/or lines are pooled.