SOD1 and amyotrophic lateral sclerosis: Phenotypically, mutant SOD1 toxicity in ALS is universally attributed to SOD1 misfolding and aggregation5, with a clear inverse correlation between mutant SOD1 misfolding and aggregation and disease duration in SOD1 patients6–8, suggesting that a gain of novel noxious function upon misfolding may be involved in the pathogenesis of ALS, as proposed previously9.