As CHCHD10 is a mitochondria-resident protein localized to the intermembrane space [50] and physically interacts with TDP-43 [17], we hypothesized that FTD/ALS-linked CHCHD10 mutations promote the accumulation of misfolded TDP-43 upon mislocalization to the cytoplasm and import into mitochondria. Here, TARDBP is linked to amyotrophic lateral sclerosis.