The gene Coiled-coil-helix-coiled-coil-helix domain containing 10 (CHCHD10) encodes a small 15-kDa mitochondrial intermembrane space (IMS) protein, in which multiple mutations have been linked to the etiology of both sporadic and familial FTD-ALS spectrum disorders [21–24]. Here, CHCHD10 is linked to amyotrophic lateral sclerosis.