To address this issue and to further understand the role of TCA cycle in ALS pathology, we co-overexpressed mitochondrial isocitrate dehydrogenase 3 (IDH3) subunit a, the catalytic domain of IDH3 (human, hIDH3a or Drosophila, dIDH3a) [15], with TDP-43 in MNs and tested its effect on TDP-43-dependent locomotor defects using larval turning assays. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.