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Cell Dev. Biol."],"abstract":"<jats:p>Coiled-coil-helix-coiled-coil-helix domain containing 2 (CHCHD2) and Coiled-coil-helix-coiled-coil-helix domain containing 10 (CHCHD10) are mitochondrial proteins that are thought to be genes which duplicated during evolution and are the causative genes for Parkinson\u2019s disease and amyotrophic lateral sclerosis\/frontotemporal lobe dementia, respectively. CHCHD2 forms a heterodimer with CHCHD10 and a homodimer with itself, both of which work together within the mitochondria. Various pathogenic and disease-risk variants have been identified; however, how these mutations cause neurodegeneration in specific diseases remains a mystery. This review focuses on important new findings published since 2019 and discusses avenues to solve this mystery.<\/jats:p>","DOI":"10.3389\/fcell.2022.996061","type":"journal-article","created":{"date-parts":[[2022,9,9]],"date-time":"2022-09-09T06:54:10Z","timestamp":1662706450000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":24,"title":["Neurodegeneration-associated mitochondrial proteins, CHCHD2 and CHCHD10\u2013what distinguishes the two?"],"prefix":"10.3389","volume":"10","author":[{"given":"Aya","family":"Ikeda","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuzuru","family":"Imai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nobutaka","family":"Hattori","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,9,9]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s10048-014-0421-1","article-title":"Mutation in the novel nuclear-encoded mitochondrial protein CHCHD10 in a family with autosomal dominant mitochondrial myopathy","volume":"16","author":"Ajroud-Driss","year":"2015","journal-title":"Neurogenetics"},{"key":"B2","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1007\/s00401-019-01989-y","article-title":"ALS\/FTD mutant CHCHD10 mice reveal a tissue-specific toxic gain-of-function and mitochondrial stress response","volume":"138","author":"Anderson","year":"2019","journal-title":"Acta Neuropathol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"440","DOI":"10.1016\/j.bbamcr.2016.11.029","article-title":"Abl2 kinase phosphorylates Bi-organellar regulator MNRR1 in mitochondria, stimulating respiration","volume":"1864","author":"Aras","year":"2017","journal-title":"Biochim. 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