On the other hand, TDP-43, an RNA/DNA binding protein linked to frontotemporal lobar degeneration and amyotrophic lateral sclerosis, was recently found to mediate prominent structural and functional damage to mitochondria along with activation of the mitochondrial unfolded protein response (UPRmt) (Wang et al., 2019). The gene discussed is TARDBP; the disease is frontotemporal dementia.