The transgene comprises a cDNA encoding full length human ATN1 containing a 118 residue polyglutamine tract, encoded by 118 CAG repeats, inserted into a transgenic vector containing the rat neuron-specific enolase promoter. This transgenic line carries one copy of the construct and, as shown by immunohistology and western blot analyses, expresses human ATN1 protein in the brain. (J:112706)