Previous work from our laboratory showed that fibroblasts from mutant-SOD1-I113T ALS patients undergo a similar switch in energy generating pathways to that observed in the TDP-43 model.141 Our results reported mitochondrial defects and reduced ATP production from oxidative phosphorylation, which, however, was accompanied by an increase in glycolytic flux. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.