Similar “starvation” phenotypes have been previously reported in both SOD1 and TDP-43 transgenic models and correspond closely to the hypolipidemic state previously observed in ALS patients.142 This parallel upregulation of glycolysis might be either a compensatory response to the loss of mitochondrial ATP synthesis, or, on the contrary, an effect of the TDP-43 and SOD1 mutations which further drives mitochondrial uncoupling and limits ATP production via the ETC. Here, SOD1 is linked to amyotrophic lateral sclerosis.