Studies in models of MJD have demonstrated an interaction between human ataxin-3 (wild-type or mutant) and transcriptional repressor histone deactylase-3 (HDAC; class I HDAC), further highlighting a role for ataxin-3 in regulating transcription [14, 15]. This evidence concerns the gene ATXN3 and Spinocerebellar ataxia type 3.