SOD1 and amyotrophic lateral sclerosis: Most recently, the Cu chelator tetrathiomolybdate (TM) was shown to significantly improve symptoms and survival in SOD-1(G93A) ALS mice (Tokuda et al., 2008) by removal of Cu from the Cys111 residue in mutant SOD-1 but not from histidyl sites essential for SOD-1 enzymatic activity, thereby preventing “aberrant copper chemistry.” Subsequently, an almost threefold accumulation of Cu in spinal cords of SOD-1(G93A) mice was reversed by TM treatment and modulations in expression of a variety of Cu-containing proteins in SOD-1 mutants were reported (Tokuda et al., 2013).