CHCHD10 and amyotrophic lateral sclerosis: Mutations in coiled-coil-helix-coiled-coil-helix domain containing 10 (CHCHD10), a nuclear encoded mitochondrial protein, are associated with both FTD and ALS.211 CHCHD10 is enriched in the cristae junctions,211 as part of the mitochondrial contact site and cristae organizing system complex.212 Initially reported as a genetic cause of ALS-FTD, further mutations in CHCHD10 have now been reported as causative for mitochondrial DNA instability disorder, late-onset spinal motor neuropathy and Charcot-Marie-Tooth disease type 2.211