SOD1 and amyotrophic lateral sclerosis: If administered to hSOD1G93A/hCCS mice during the pre‐symptomatic disease phase CuII(atsm) prevented the development of ALS altogether,[154] whilst administration to hSOD1G93A and hSOD1G37R mice after symptom onset markedly improved locomotor function and survival time.[228, 230] Importantly, the therapeutic benefit of this compound across numerous mutant SOD1 transgenic mouse strains has been independently verified by multiple research groups.[154, 231] Accordingly, phase II/III clinical trials of CuII(atsm) have recently begun in a small cohort of ALS patients (n=80; Table 3).