SOD1 and amyotrophic lateral sclerosis: Restricted expression of human SOD1 variants (wild-type-, G37R- and G93A-hSOD1) in the skeletal muscle of mice recapitulated an ALS-type phenotype including age related neurological symptoms, skeletal muscle pathology, including limb weakness, muscle atrophy, myofiber cell death and NMJ abnormalities, as well as MN neuronopathy/distal axonopathy [43,44].