SOD1 and amyotrophic lateral sclerosis: On this matter, previous studies reported that NOX2 activates the NF-κB system in ALS microglia (36) in turn upregulating NOX2 (37), and that inactivation of NOX2 decreases reactive oxygen species production and extends survival of SOD1-G93A mice, thus proposing NOX2 as a gene modifier in ALS (38).