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In mammals, it serves as the electron acceptor for nine mitochondrial inner membrane dehydrogenases. These include the NADH dehydrogenase (complex I, CI) and succinate dehydrogenase (complex II, CII) but also several others that are often omitted in the context of respiratory enzymes: dihydroorotate dehydrogenase, choline dehydrogenase, electron\u2010transferring flavoprotein dehydrogenase, mitochondrial glycerol\u20103\u2010phosphate dehydrogenase, proline dehydrogenases 1 and 2, and sulfide:quinone oxidoreductase. The metabolic pathways these enzymes are involved in range from amino acid and fatty acid oxidation to nucleotide biosynthesis, methylation, and hydrogen sulfide detoxification, among many others. The CoQ\u2010linked metabolism depends on CoQ reoxidation by the mitochondrial complex III (cytochrome\n                    <jats:italic>\n                      bc\n                      <jats:sub>1<\/jats:sub>\n                    <\/jats:italic>\n                    complex, CIII). However, the literature is surprisingly limited as for the role of the CoQ\u2010linked metabolism in the pathogenesis of human diseases of oxidative phosphorylation (OXPHOS), in which the CoQ homeostasis is directly or indirectly affected. In this review, we give an introduction to CIII function, and an overview of the pathological consequences of CIII dysfunction in humans and mice and of the CoQ\u2010dependent metabolic processes potentially affected in these pathological states. Finally, we discuss some experimental tools to dissect the various aspects of compromised CoQ oxidation.\n                  <\/jats:p>","DOI":"10.1111\/febs.16164","type":"journal-article","created":{"date-parts":[[2021,8,24]],"date-time":"2021-08-24T12:25:02Z","timestamp":1629807902000},"page":"6936-6958","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":137,"title":["The mitochondrial coenzyme Q junction and complex III: biochemistry and pathophysiology"],"prefix":"10.1111","volume":"289","author":[{"given":"Rishi","family":"Banerjee","sequence":"first","affiliation":[{"name":"Folkh\u00e4lsan Research Center  Helsinki Finland"},{"name":"Stem Cells and Metabolism Research Program Faculty of Medicine University of Helsinki  Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janne","family":"Purhonen","sequence":"additional","affiliation":[{"name":"Folkh\u00e4lsan Research Center  Helsinki 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