ATXN3 and amyotrophic lateral sclerosis: Based on our previous neurodegeneration models (ALS models), we expressed human ATXN3, either wild-type ATXN3-CAG10 (Fig. 1A) or mutant ATXN3-CAG89 (Fig. 1B), in the worm's 26 GABAergic motoneurons using the promoter for the gene unc-47, which encodes a vesicular GABA transporter (Vaccaro et al., 2012a).