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Of all the factors involved in ALS, mitochondria have been considered as a major player, as secondary mitochondrial dysfunction has been found in various models and patients. Abnormal mitochondrial morphology, defects in mitochondrial dynamics, altered activities of respiratory chain enzymes and increased production of reactive oxygen species have been described. Moreover, the identification of CHCHD10 variants in ALS patients was the first genetic evidence that a mitochondrial defect may be a primary cause of MN damage and directly links mitochondrial dysfunction to the pathogenesis of ALS. In this review, we focus on the role of mitochondria in ALS and highlight the pathogenic variants of ALS genes associated with impaired mitochondrial functions. The multiple pathways demonstrated in ALS pathogenesis suggest that all converge to a common endpoint leading to MN loss. This may explain the disappointing results obtained with treatments targeting a single pathological process. Fighting against mitochondrial dysfunction appears to be a promising avenue for developing combined therapies in the future.<\/jats:p>","DOI":"10.3390\/genes14111981","type":"journal-article","created":{"date-parts":[[2023,10,24]],"date-time":"2023-10-24T06:28:25Z","timestamp":1698128905000},"page":"1981","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":60,"title":["Mitochondria, a Key Target in Amyotrophic Lateral Sclerosis Pathogenesis"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3039-7101","authenticated-orcid":false,"given":"Emmanuelle C.","family":"Genin","sequence":"first","affiliation":[{"name":"Institute for Research on Cancer and Aging, Nice (IRCAN), Universit\u00e9 C\u00f4te d\u2019Azur, Inserm U1081, CNRS UMR7284, Centre Hospitalier Universitaire (CHU) de Nice, 06200 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e9lanie","family":"Abou-Ali","sequence":"additional","affiliation":[{"name":"Institute for Research on Cancer and Aging, Nice (IRCAN), Universit\u00e9 C\u00f4te d\u2019Azur, Inserm U1081, CNRS UMR7284, Centre Hospitalier Universitaire (CHU) de Nice, 06200 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V\u00e9ronique","family":"Paquis-Flucklinger","sequence":"additional","affiliation":[{"name":"Institute for Research on Cancer and Aging, Nice (IRCAN), Universit\u00e9 C\u00f4te d\u2019Azur, Inserm U1081, CNRS UMR7284, Centre Hospitalier Universitaire (CHU) de Nice, 06200 Nice, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2084","DOI":"10.1016\/S0140-6736(17)31287-4","article-title":"Amyotrophic Lateral Sclerosis","volume":"390","author":"Hardiman","year":"2017","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"17071","DOI":"10.1038\/nrdp.2017.71","article-title":"Amyotrophic Lateral Sclerosis","volume":"3","author":"Hardiman","year":"2017","journal-title":"Nat. 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