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Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2018,8,7]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Inherited pathogenic mutations in genes required for copper delivery to cytochrome\n                    <jats:italic>c<\/jats:italic>\n                    oxidase (CcO) perturb mitochondrial energy metabolism and result in fatal mitochondrial disease. A prior attempt to treat human patients with these mutations by direct copper supplementation was not successful, possibly because of inefficient copper delivery to the mitochondria. We performed a targeted search to identify compounds that can efficiently transport copper across biological membranes and identified elesclomol (ES), an investigational anticancer drug, as the most efficient copper delivery agent. ES rescues CcO function in yeast, zebrafish, and mammalian models of copper deficiency by increasing cellular and mitochondrial copper content. 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L.","family":"Chan","sequence":"additional","affiliation":[{"name":"Department of Drug Discovery and Biomedical Sciences, Medical University of South Carolina, Charleston, SC 29425"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8488-7822","authenticated-orcid":false,"given":"Scot C.","family":"Leary","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, University of Saskatchewan, Saskatoon, SK S7N 5E5, Canada;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Byung-Eun","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Animal and Avian Sciences, University of Maryland, College Park, MD 20742;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D.","family":"Gitlin","sequence":"additional","affiliation":[{"name":"Eugene Bell Center for Regenerative Biology and Tissue Engineering, Marine Biological Laboratory, Woods Hole, MA 02543;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9920-2563","authenticated-orcid":false,"given":"Vishal M.","family":"Gohil","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Biophysics, Texas A&amp;M University, College Station, TX 77843;"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2018,7,23]]},"reference":[{"key":"e_1_3_3_1_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-micro-030117-020444"},{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.semcdb.2017.08.055"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1039\/C7MT00221A"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M113.470674"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M312693200"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M606839200"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M404747200"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.275.1.619"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddh197"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddv503"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cmet.2015.04.012"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddv265"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.E14-11-1526"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1038\/15513"},{"key":"e_1_3_3_15_2","first-page":"1104","article-title":"Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy","volume":"67","author":"Valnot I","year":"2000","unstructured":"I Valnot, , Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy. 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