Spinocerebellar ataxia type 1 (SCA1)  Transgenic mouse with human ATXN1 with 82 CAG trinucleotide repeats under control of a Purkinje-cell promoter  TUDCA  Subcutaneous injection  500 mg/kg  TUDCA crossed the blood-brain barrier but had no effect on cell survival  Kaemmerer et al., 2001