As in our LV-based model of SCA7, several SCA7 rodent models [14, 15], which replicate many features of the human condition, and brains from SCA7 patients [11, 44] display abundant inclusions that consistently stain positively for proteasome subunits, ubiquitin, and molecular chaperones. This evidence concerns the gene ATXN7 and spinocerebellar ataxia 7.