In addition to rare mutations and common SNPs, a recent publication reported that ATXN2 dysfunction influences the TDP-43-dependent toxicity seen in ALS and that the intermediate-length expansions to 27–33 triplets in the ATXN2 polyglutamine (polyQ) region act as ALS risk factors in 4.7% of North American patients [14]. Here, ATXN2 is linked to amyotrophic lateral sclerosis.