SOD1 and amyotrophic lateral sclerosis: As mutations in this gene represent indeed almost 20% of familial cases, murine models carrying a SOD1-gene mutation, such as the SOD1-G93A mice, can provide insights into ALS pathogenesis, as they present an ALS-like phenotype which includes mitochondrial dysfunctions, SOD1 aggregation, motor neuron death, and paralysis [2].