TDP-43 and FTD/ALS-linked CHCHD10 mutations destabilized OPA1-mitofilin complex, impairing mitochondrial fusion and respiration in brains of human FTLD-TDP (frontotemporal lobar degeneration with TDP-43 inclusions) patients, TDP-43 transgenic mice, and HEK293T cells (Liu T. et al., 2020). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.