Increasing evidence indicates that a toxic gain-of-function in SOD1 mutants is a fundamental mechanism for the pathogenesis of ALS (Williamson et al., 2000; Deng et al., 2006; Sau et al., 2007; Rotunno and Bosco, 2013; Bunton-Stasyshyn et al., 2015; Silverman et al., 2016). Here, SOD1 is linked to amyotrophic lateral sclerosis.