Specifically, the aggregation of mutated SOD1 at the level of the mitochondrial intermembrane space reduces the activity of the electron transport chain; in addition, the protein aggregates, resulting in the inhibition of voltage-gated anion channel 1 (VDAC1) activity, ADP permeability, and increased ALS progression [52,53]. Here, SOD1 is linked to amyotrophic lateral sclerosis.