ATXN2 and amyotrophic lateral sclerosis: As overexpression of the human TDP-43 protein in mice can result in a phenotype reminiscent of ALS that can be attenuated by the knockout of its ataxin-2 gene26, we initially sought to target the mouse ataxin-2 (mATXN2) transcript to ensure that this strategy could be assessed in a validated rodent model.