Using hiPSCs derived from ALS patients with TDP-43 mutations, Dafinca and co-workers (2024) [214] showed an interaction between TDP-43 and ATP synthase and COX5A leading to reduced mitochondrial respiration and ATP production; mitochondrial dysfunction plays an important role in MN degeneration in ALS. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.