SOD1 and amyotrophic lateral sclerosis: Several transgenic mouse models have been created based on identified gene mutations, and the most frequently used models include mutations in SOD1, TARDBP, and FUS. The most well-characterized ALS model is the Sod1G93A mouse that shows a progressive muscle wasting phenotype from early to terminal stages, although this model only recapitulates lower motor neuron symptoms (8).