SOD1 and amyotrophic lateral sclerosis: Furthermore, the potential to improve the symptoms of ALS and protect motor neurons in the CNS via therapeutic strategies that modulate Cu bioavailability has already been demonstrated24; treating mutant SOD1 mice with Cu-chelating agents such as ammonium tetrathiomolybdate and D-penicillamine or with the Cu-delivery agent CuII(atsm) improves their neurological phenotype and extends survival7, 8, 9, 10, 22, 25, 26, 27, 28.