SOD1 and amyotrophic lateral sclerosis: In contrast, it has been found that mutations of SOD1 increase its denytrosylation activity, and denytrosylation of many mitochondrial proteins can hamper the normal homeostasis in mitochondria, ultimately leading to cell death in ALS models, whilst addition of SNO-donor compounds to the mutant SOD1-containing cells prevents this course of events [184].