SOD1 and amyotrophic lateral sclerosis: Alongside inflammation, ROS accumulation substantially damages MNs and weakens the BCNSB in ALS.79 In vitro blood brain barriers developed from SOD1-G93A ALS models show increased ROS production, Nrf2, and NF-kB activation in endothelial cells due to mutant astrocyte signaling.80 In rats, pre-ALS and ALS brains have elevated nitrilation, superoxide production, lipid peroxidation, and manganese superoxide dismutase activity, all markers of elevated oxidative stress.