SOD1 and amyotrophic lateral sclerosis: Further, we demonstrated that excess cupric ions not only induced the oxidation of either apo A4V or Zn2-A4V and triggered the aggregation of oxidized A4V under copper-mediated oxidative conditions, but also triggered the aggregation of non-oxidized A4V, and thus provided a plausible model to explain how pathological SOD1 mutants misfold in ALS-affected motor neurons.