SOD1 and amyotrophic lateral sclerosis: In ALS, mutSOD1 associates more strongly with Rac1 compared to the wild type form of SOD1 (SOD1G93A vs. SOD1WT transgenic mice), being less sensitive to redox uncoupling, consequently leading to the hyperactivation of NOX-derived O2•- by endomembranes [114] (Figure 2).