SOD1 and neurodegenerative disease: Forced expression in mice of a human-mutated form of SOD1 (the mutant SOD1 G93A) resulted in the generation of transgenic mice [49] that recapitulates the pathogenic phenotype of ALS patients, namely, an adult onset neurodegenerative disease that is characterized by locomotor impairments, spinal cord and muscular atrophy, motor neuron loss, changes at the NMJ, muscle denervation, and premature death.