ATXN2 [13,14] is a 140 kDa protein that has mainly been studied as a polyglutamine repeat protein in the context of spinocerebellar ataxia type 2 (SCA2) [15] and amyotrophic lateral sclerosis (ALS) [16,17,18,19]. This evidence concerns the gene ATXN2 and spinocerebellar ataxia type 2.