SOD1 and amyotrophic lateral sclerosis: Mutant SOD1G93A transgenic mouse line is the most well-characterized preclinical ALS model because it recapitulates key disease features such as progressive decrease of motor performance, neuromuscular junction (NMJ) denervation, loss of spinal motoneurons concomitantly with astrogliosis and microgliosis, organelle dysfunction and presence of intracellular mutant SOD1 inclusions [5, 6].