More than 100 different mutations in the gene encoding Cu, Zn-superoxide dismutase
(SOD1) cause preferential motor neuron degeneration in familial amyotrophic lateral
sclerosis (ALS). Although the cellular target (s) of mutant SOD1 toxicity have not been
precisely specified, evidence to date supports the hypothesis that ALS-related mutations
may increase the burden of partially unfolded SOD1 species. Influences that may destabilize
SOD1 in vivo include impaired metal ion binding, reduction of the intrasubunit disulfide …