In a transgenic mouse model, selective expression of mutated SOD1 G93A (Dobrowolny et al., 2008) or G37R (Wong and Martin, 2010) exclusively in the skeletal muscle demonstrated progressive muscle atrophy, associated with a significant reduction in muscle strength, alterations in the contractile apparatus-sarcomere and sarcotubular system disorganization, and mitochondrial dysfunction, mimicking a pathologic phenotype consistent with ALS. Here, SOD1 is linked to amyotrophic lateral sclerosis.