Presumably, the most convincing argument supporting pathological involvement of iron accumulation and iron-mediated oxidative stress in the progression of ALS derives from studies showing beneficial effects of iron chelation therapy in transgenic mice overexpressing human mutated SOD1 gene (Jeong et al., 2009; Kupershmidt et al., 2009; Wang et al., 2011; Lee et al., 2015). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.