SOD1 and amyotrophic lateral sclerosis: In skeletal muscle from SOD1G93A transgenic mice, a model that expresses a mutant SOD1 carrying the G93A missense mutation and which develops an ALS-like motor neuron disease [79], the reduced mitochondrial dynamics and network were associated with increased ROS generation [81], in the absence of motor neuron axonal withdrawal, suggesting that muscle mitochondria could be directly affected by a mutation localized into the skeletal muscle [82].