Transgenic SOD1G93A (Wang et al., 2011; Lee et al., 2015) and SOD1G37R (Jeong et al., 2009) mice, abundantly expressing active mutant SOD1 and showing fast and slow progression of the disease, respectively, are the most intensely studied in vivo models for investigating the contribution of iron to the pathogenesis of ALS. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.