SOD1 and amyotrophic lateral sclerosis: ALS was initially thought to be associated with oxidative stress, as it was first shown to be associated with the mutant SOD1, TDP-43 or other ALS-related mutant proteins that can all lead to mitochondrial imbalance in ALS and affect mitochondrial respiration as well as ATP production, calcium handling, mitochondrial dynamics and apoptotic signaling [204, 205].