SOD1 and amyotrophic lateral sclerosis: Misfolding and self-assembly of SOD1 turned into neurotoxic oligomers and aggregates gain toxic function, leading to mitochondrial dysfunction, dysregulation of the ubiquitin-proteasome system, disruption of cytoskeletal elements, and sequestration of essential proteins (4, 5, 6), which demonstrates the pathological importance of SOD1 misfolding and aggregation in ALS.