SOD1 and amyotrophic lateral sclerosis: The phenotypic hallmark of the SOD1-dependent ALS is the presence of amyloid aggregates of SOD1 in affected tissues, which are the ultimate outcome of SOD1 misfolding – the product of a gradual destabilization of the SOD1 structure due to the loss of metal ions and the reduction of the stabilizing intrasubunit disulfide bond10–12.