SOD1 and amyotrophic lateral sclerosis: We have previously demonstrated that muscle-specific expression of SOD1 mutation in the MLC/SOD1G93A transgenic mouse model, which selectively expresses the mutant SOD1G93A gene in the skeletal muscle under the transcriptional control of a muscle-specific promoter (MLC), induces accumulation of Reactive Oxygen Species, causes muscle atrophy with a concomitant alteration in the ultrastructure and in the functional performance of skeletal muscles, and promotes microglia activation in the spinal cord, that is a presymptomatic sign of ALS disease (Dobrowolny et al., 2008b).