TUDCA treatment was used to determine whether the survival of Purkinje cells and the onset and progression of ataxia was altered in a transgenic mouse model of SCA1, in which 30 copies of the human ATXN1 cDNA containing a CAG trinucleotide repeat of 82 repeats was under the control of the Pcp-2 Purkinje-cell specific promoter l (Kaemmerer et al., 2001). The gene discussed is ATXN1; the disease is cerebellar ataxia.