Interestingly, polyglutamine (polyQ) intermediate expansions (24–34 CAG repeats) in the neuronal stress protein Ataxin 1 (ATXN-1) and Ataxin 2 (ATXN-2) genes have been recently reported to be independently associated with an increased risk for ALS [62, 63]. This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.