{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T17:55:20Z","timestamp":1792000520381,"version":"4.4.0"},"reference-count":60,"publisher":"Oxford University Press (OUP)","issue":"19","license":[{"start":{"date-parts":[[2020,9,25]],"date-time":"2020-09-25T00:00:00Z","timestamp":1600992000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"National Institute of Health","award":["P01 HD080642-01"],"award-info":[{"award-number":["P01 HD080642-01"]}]},{"DOI":"10.13039\/501100006393","name":"University of Granada","doi-asserted-by":"publisher","award":["UCE-PP2017-06"],"award-info":[{"award-number":["UCE-PP2017-06"]}],"id":[{"id":"10.13039\/501100006393","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["MDA-602322"],"award-info":[{"award-number":["MDA-602322"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Ministerio de Ciencia e Innovaci\u00f3n, Spain, and the ERDF","award":["RTI2018-093503-B-100"],"award-info":[{"award-number":["RTI2018-093503-B-100"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,11,25]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Abnormalities of one carbon, glutathione and sulfide metabolisms have recently emerged as novel pathomechanisms in diseases with mitochondrial dysfunction. However, the mechanisms underlying these abnormalities are not clear. Also, we recently showed that sulfide oxidation is impaired in Coenzyme Q10 (CoQ10) deficiency. This finding leads us to hypothesize that the therapeutic effects of CoQ10, frequently administered to patients with primary or secondary mitochondrial dysfunction, might be due to its function as cofactor for sulfide:quinone oxidoreductase (SQOR), the first enzyme in the sulfide oxidation pathway. Here, using biased and unbiased approaches, we show that supraphysiological levels of CoQ10 induces an increase in the expression of SQOR in skin fibroblasts from control subjects and patients with mutations in Complex I subunits genes or CoQ biosynthetic genes. This increase of SQOR induces the downregulation of the cystathionine \u03b2-synthase and cystathionine \u03b3-lyase, two enzymes of the transsulfuration pathway, the subsequent downregulation of serine biosynthesis and the adaptation of other sulfide linked pathways, such as folate cycle, nucleotides metabolism and glutathione system. These metabolic changes are independent of the presence of sulfur aminoacids, are confirmed in mouse models, and are recapitulated by overexpression of SQOR, further proving that the metabolic effects of CoQ10 supplementation are mediated by the overexpression of SQOR. Our results contribute to a better understanding of how sulfide metabolism is integrated in one carbon metabolism and may explain some of the benefits of CoQ10 supplementation observed in mitochondrial diseases.<\/jats:p>","DOI":"10.1093\/hmg\/ddaa214","type":"journal-article","created":{"date-parts":[[2020,9,23]],"date-time":"2020-09-23T19:13:28Z","timestamp":1600888408000},"page":"3296-3311","source":"Crossref","is-referenced-by-count":34,"title":["Coenzyme Q10 modulates sulfide metabolism and links the mitochondrial respiratory chain to pathways associated to one carbon metabolism"],"prefix":"10.1093","volume":"29","author":[{"given":"Pilar","family":"Gonz\u00e1lez-Garc\u00eda","sequence":"first","affiliation":[{"name":"Instituto de Biotecnolog\u00eda, Centro de Investigaci\u00f3n Biom\u00e9dica, Universidad de Granada, Granada 18016, Spain"},{"name":"Departamento de Fisiolog\u00eda, Facultad de Medicina, Universidad de Granada, Granada 18016, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6414-6859","authenticated-orcid":false,"given":"Agust\u00edn","family":"Hidalgo-Guti\u00e9rrez","sequence":"additional","affiliation":[{"name":"Instituto de Biotecnolog\u00eda, Centro de Investigaci\u00f3n Biom\u00e9dica, Universidad de Granada, Granada 18016, Spain"},{"name":"Departamento de Fisiolog\u00eda, Facultad de Medicina, Universidad de Granada, Granada 18016, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Mascaraque","sequence":"additional","affiliation":[{"name":"Departamento de Fisiolog\u00eda, Facultad de Medicina, Universidad de Granada, Granada 18016, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eliana","family":"Barriocanal-Casado","sequence":"additional","affiliation":[{"name":"Instituto de Biotecnolog\u00eda, Centro de Investigaci\u00f3n Biom\u00e9dica, Universidad de Granada, Granada 18016, Spain"},{"name":"Departamento de Fisiolog\u00eda, Facultad de Medicina, Universidad de Granada, Granada 18016, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammed","family":"Bakkali","sequence":"additional","affiliation":[{"name":"Departamento de Gen\u00e9tica, Facultad de Ciencias, Universidad de Granada, Granada 18071, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcello","family":"Ziosi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Columbia University Medical Center, New York 10032, NY, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ussipbek Botagoz","family":"Abdihankyzy","sequence":"additional","affiliation":[{"name":"Department of Biophysics and Biomedicine, Al-Farabi Kazakh National University, Almaty 050040, Kazakhstan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabina","family":"S\u00e1nchez-Hern\u00e1ndez","sequence":"additional","affiliation":[{"name":"Genomic Medicine Department, Centre for Genomics and Oncological Research, Granada 18007, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Germaine","family":"Escames","sequence":"additional","affiliation":[{"name":"Instituto de Biotecnolog\u00eda, Centro de Investigaci\u00f3n Biom\u00e9dica, Universidad de Granada, Granada 18016, Spain"},{"name":"Departamento de Fisiolog\u00eda, Facultad de Medicina, Universidad de Granada, Granada 18016, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Holger","family":"Prokisch","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, Technische Universit\u00e4t M\u00fcnchen, M\u00fcnchen 81675, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisco","family":"Mart\u00edn","sequence":"additional","affiliation":[{"name":"Genomic Medicine Department, Centre for Genomics and Oncological Research, Granada 18007, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catarina M","family":"Quinzii","sequence":"additional","affiliation":[{"name":"Department of Neurology, Columbia University Medical Center, New York 10032, NY, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis C","family":"L\u00f3pez","sequence":"additional","affiliation":[{"name":"Instituto de Biotecnolog\u00eda, Centro de Investigaci\u00f3n Biom\u00e9dica, Universidad de Granada, Granada 18016, Spain"},{"name":"Departamento de Fisiolog\u00eda, Facultad de Medicina, Universidad de Granada, Granada 18016, 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