Given that perturbations to SOD1 metalation appear to be restricted to the central nervous system in mammalian models of ALS,[157] no outcome measures for any of the current clinical trials of CuII(atsm) in familial and sporadic ALS patients involve measurement of SOD1 metalation, protein levels or catalytic activity, and thus any association between therapeutic benefit and alterations to SOD1 biochemistry can only be inferred from these trials. Here, SOD1 is linked to amyotrophic lateral sclerosis.