These findings address several very important issues (i) importance of CHCHD10 for mitochondrial respiration in motoneurons (ii) confirmation of CHCHD10 mutations association with ALS and FTD spectrum (iii) recognition of mitochondrial protein import impairment as possible pathogenic mechanism in disease progression (iv) development of “epigenetic boosting” therapies targeting expression of proteins involved mitochondrial protein import machinery and/or CHCHD10 (to overcome haploinsufficiency). This evidence concerns the gene CHCHD10 and amyotrophic lateral sclerosis.