The apparent incongruous results derived from CuII(atsm) and treatments aimed at mitigating copper accumulation in the CNS may, therefore, illustrate the important role that the metalation state of SOD1 plays in its contribution to neuronal death in ALS, with both strategies preventing accumulation of an aberrant, partially metallated intermediary. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.