This transgenic strain contains human ATXN3 (MJD1, polyQ repeat 64) gene fragment driven by rat Eno2 (NSE: neuron specific enolase) promoter. Transgene ATXN3*64Q is the construct containing of C-terminal part and polyglutamine part of human ATXN3, but not of N-terminal part. Aggregation of ATXN3*64Q polygultamine leads to ataxia. Line 16 is more likely to develop ataxia than Line 29.