SOD1 and amyotrophic lateral sclerosis: Of note, Nox enzymes control relevant proinflammatory signaling pathways involved in the progression of ALS, such as those mediated by IL-1β and TNF-α via redox-dependent activation of NF-κB. Finally, marked elevation of 2-thiobarbituric reactive substances in plasma (i.e., MDA) was found both in mutant SOD-1 mice and in patients with sporadic ALS [62, 63].