Similar to findings in HD models, early findings in MJD saw correlations between the expression of truncated, polyQ stretch-containing forms of the disease protein Atx3 and increased toxicity, which was accompanied by nuclear mislocalization and aggregation in vitro and in vivo (Ikeda et al., 1996; Paulson et al., 1997; Goti et al., 2004; Haacke et al., 2006). This evidence concerns the gene ATXN3 and Huntington disease.