SOD1 and amyotrophic lateral sclerosis: While reducing SOD1 levels directly in the muscles of mutSOD1 transgenic mice did not affect the onset of the disease or survival (Miller et al. 2006) muscle-restricted expression of mutSOD1 led to alterations associated with ALS pathogenesis (Dobrowolny et al. 2009) and a classic ALS mouse phenotype (Dobrowolny et al. 2009; Wong and Martin 2010; Maimon et al. 2018).