Previously, we demonstrated that genetic ablation of Als2 in SOD1H46R, but not SOD1G93A, mice on a B6 background aggravated the mutant SOD1-associated disease symptoms and led to the earlier death [18], suggesting distinctive susceptibilities to ALS2 loss in different mutant SOD1-expressing ALS mouse models. Here, ALS2 is linked to amyotrophic lateral sclerosis.