The CHCHD10 gene encoding a mitochondrial protein (coiled-coil-helix-coiled-coil-helix domain containing 10) is mutated in familial and sporadic FTD, ALS, and mixed FTD–ALS [11,12,13,14,15,16], linking CHCHD10 dysfunction to the etiology of these disorders. Here, CHCHD10 is linked to amyotrophic lateral sclerosis.