Disulfide scrambling in superoxide dismutase 1 reduces its cytotoxic effect in cultured cells and promotes protein aggregation.
Mutations in the gene coding for superoxide dismutase 1 (SOD1) are associated with familiar forms of the neurodegenerative disease amyotrophic lateral sclerosis (ALS).These mutations are believed to result in a "gain of toxic function", leading to neuronal degeneration.The exact mechanism is still unknown, but misfolding/aggregation events are gene