{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T01:42:53Z","timestamp":1791250973449,"version":"4.1.0"},"reference-count":53,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2025,4,29]],"date-time":"2025-04-29T00:00:00Z","timestamp":1745884800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,6,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>The copper ionophore disulfiram (DSF) is commonly used to treat chronic alcoholism and has potential anti-cancer activity. Using a yeast-based screening assay of FDA-approved compounds, DSF was herein identified for its ability to improve oxidative phosphorylation-dependent growth of various yeast models of mitochondrial diseases caused by a wide range of defects in ATP synthase, complexes III and IV, cardiolipin remodeling, maintenance and translation of the mitochondrial genome. This compound also showed beneficial effects in cells derived from patients suffering from Barth or MELAS syndromes, two mitochondrial diseases associated respectively with a lack in cardiolipin remodeling and protein synthesis inside the organelle. We provide evidence that the rescuing activity of DSF results from its ability to transport copper ions across biological membranes. Indeed, other copper ionophores (pyrithione and elesclomol) and supplementation of the growth media with copper ions had also beneficial effects in yeast and human cells with dysfunctional mitochondria. Our data suggest that the copper-dependent rescuing activity in these cells results from a better capacity to assemble cytochrome c oxidase. Altogether, our findings hold promise for the development of new therapeutic strategies for mitochondrial disorders.<\/jats:p>","DOI":"10.1093\/hmg\/ddaf061","type":"journal-article","created":{"date-parts":[[2025,4,29]],"date-time":"2025-04-29T12:01:45Z","timestamp":1745928105000},"page":"1072-1085","source":"Crossref","is-referenced-by-count":6,"title":["The copper ionophore disulfiram improves mitochondrial function in various yeast and human cellular models of mitochondrial diseases"],"prefix":"10.1093","volume":"34","author":[{"given":"Claire","family":"Almyre","sequence":"first","affiliation":[{"name":"Univ. Bordeaux, CNRS, IBGC, UMR 5095 , Bordeaux F-33000 ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nolwenn","family":"Bounaix","sequence":"additional","affiliation":[{"name":"Univ Angers, CHU Angers, INSERM1083, CNRS6015, MITOVASC, MITOLAB, SFR ICAT , Angers F-49000 ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fran\u00e7ois","family":"Godard","sequence":"additional","affiliation":[{"name":"Univ. Bordeaux, CNRS, IBGC, UMR 5095 , Bordeaux F-33000 ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier R","family":"Baris","sequence":"additional","affiliation":[{"name":"Univ Angers, CHU Angers, INSERM1083, CNRS6015, MITOVASC, MITOLAB, SFR ICAT , Angers F-49000 ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Louise","family":"Cayer","sequence":"additional","affiliation":[{"name":"Univ. 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therapy","volume":"41","author":"Zheng","year":"2022","journal-title":"J Exp Clin Cancer Res"},{"key":"2025060505164932900_ref29","doi-asserted-by":"crossref","first-page":"7614","DOI":"10.1128\/MCB.22.21.7614-7621.2002","article-title":"Mammalian copper chaperone Cox17p has an essential role in activation of cytochrome C oxidase and embryonic development","volume":"22","author":"Takahashi","year":"2002","journal-title":"Mol Cell Biol"},{"key":"2025060505164932900_ref30","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1097\/CMR.0b013e328334131d","article-title":"Disulfiram induces copper-dependent stimulation of reactive oxygen species and activation of the extrinsic apoptotic pathway in melanoma","volume":"20","author":"Morrison","year":"2010","journal-title":"Melanoma Res"},{"key":"2025060505164932900_ref31","doi-asserted-by":"crossref","DOI":"10.3390\/cells8040287","article-title":"Mitochondrial biogenesis and mitochondrial reactive oxygen species (ROS): a complex relationship regulated by the cAMP\/PKA Signaling pathway","volume":"8","author":"Bouchez","year":"2019","journal-title":"Cells"},{"key":"2025060505164932900_ref32","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1016\/j.bbabio.2014.02.023","article-title":"The role of mitochondrial biogenesis and ROS in the control of energy supply in proliferating cells","volume":"1837","author":"Yoboue","year":"2014","journal-title":"Biochim Biophys Acta"},{"key":"2025060505164932900_ref33","doi-asserted-by":"crossref","first-page":"1019","DOI":"10.1016\/j.bbadis.2012.01.010","article-title":"Metabolically induced heteroplasmy shifting and l-arginine treatment reduce the energetic defect in a neuronal-like model of MELAS","volume":"1822","author":"Desquiret-Dumas","year":"2012","journal-title":"Biochim Biophys Acta"},{"key":"2025060505164932900_ref34","doi-asserted-by":"crossref","first-page":"9215","DOI":"10.1002\/anie.202000451","article-title":"Superoxide dismutase 1 in health and 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Clin Med"},{"key":"2025060505164932900_ref38","doi-asserted-by":"crossref","first-page":"3615","DOI":"10.1038\/s41467-022-31413-1","article-title":"Coordination of metal center biogenesis in human cytochrome c oxidase","volume":"13","author":"Nyvltova","year":"2022","journal-title":"Nat Commun"},{"key":"2025060505164932900_ref39","doi-asserted-by":"crossref","first-page":"4114","DOI":"10.1016\/j.celrep.2019.11.054","article-title":"COA6 is structurally tuned to function as a thiol-Disulfide oxidoreductase in copper delivery to mitochondrial cytochrome c oxidase","volume":"29","author":"Soma","year":"2019","journal-title":"Cell Rep"},{"key":"2025060505164932900_ref40","doi-asserted-by":"crossref","first-page":"8161","DOI":"10.1073\/pnas.1806296115","article-title":"Elesclomol restores mitochondrial function in genetic models of copper deficiency","volume":"115","author":"Soma","year":"2018","journal-title":"Proc Natl Acad Sci 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Biochim Biophys Acta rev","volume":"1879","author":"Zhang","year":"2024","journal-title":"Cancer"},{"key":"2025060505164932900_ref49","doi-asserted-by":"crossref","first-page":"49397","DOI":"10.1074\/jbc.M207219200","article-title":"A pathogenic cytochrome b mutation reveals new interactions between subunits of the mitochondrial bc1 complex","volume":"277","author":"Saint-Georges","year":"2002","journal-title":"J Biol Chem"},{"key":"2025060505164932900_ref50","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1046\/j.1365-313X.1992.t01-38-00999.x","article-title":"Complementation of Saccharomyces cerevisiae auxotrophic mutants by Arabidopsis thaliana cDNAs","volume":"2","author":"Minet","year":"1992","journal-title":"Plant J"},{"key":"2025060505164932900_ref51","doi-asserted-by":"crossref","first-page":"680","DOI":"10.1038\/227680a0","article-title":"Cleavage of structural proteins during the assembly of the head of bacteriophage T4","volume":"227","author":"Laemmli","year":"1970","journal-title":"Nature"},{"key":"2025060505164932900_ref52","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1186\/s13104-015-1000-8","article-title":"Limitations of the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyl-2H-tetrazolium bromide (MTT) assay when compared to three commonly used cell enumeration assays","volume":"8","author":"Tonder","year":"2015","journal-title":"BMC Res Notes"},{"key":"2025060505164932900_ref53","doi-asserted-by":"crossref","first-page":"1707","DOI":"10.1016\/j.bbamcr.2008.04.018","article-title":"The optimized allotopic expression of ND1 or ND4 genes restores respiratory chain complex I activity in fibroblasts harboring mutations in these genes","volume":"1783","author":"Bonnet","year":"2008","journal-title":"Biochim Biophys Acta"}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/hmg\/article-pdf\/34\/12\/1072\/63027238\/ddaf061.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/hmg\/article-pdf\/34\/12\/1072\/63027238\/ddaf061.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,6,5]],"date-time":"2025-06-05T09:17:03Z","timestamp":1749115023000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article\/34\/12\/1072\/8121691"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4,29]]},"references-count":53,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2025,4,29]]},"published-print":{"date-parts":[[2025,6,4]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddaf061","relation":{},"ISSN":["0964-6906","1460-2083"],"issn-type":[{"value":"0964-6906","type":"print"},{"value":"1460-2083","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2025,6,15]]},"published":{"date-parts":[[2025,4,29]]}}}