SOD1 and amyotrophic lateral sclerosis: Thus the published studies support the idea that various factors may contribute to the enhancing effect of ALS mutations on amyloid formation at acidic pH: Metal-binding-region mutations may facilitate release of bound metals; both metal-binding-region and dimer-interface mutants may enhance dimer dissociation; and wild-type-like mutations in both the beta barrel and the dimer interface may facilitate the unfolding of apo-SOD1.