{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T05:40:01Z","timestamp":1785476401608,"version":"3.56.0"},"reference-count":36,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2017,9,1]],"date-time":"2017-09-01T00:00:00Z","timestamp":1504224000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2017,1,18]],"date-time":"2017-01-18T00:00:00Z","timestamp":1484697600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000272","name":"NIHR","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Manackerman Charitable Trust Fund"},{"name":"Hadassah Compensatory Fund"},{"name":"AFHU"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Mitochondrion"],"published-print":{"date-parts":[[2017,9]]},"DOI":"10.1016\/j.mito.2017.01.004","type":"journal-article","created":{"date-parts":[[2017,1,16]],"date-time":"2017-01-16T08:51:09Z","timestamp":1484556669000},"page":"36-42","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":60,"special_numbering":"C","title":["Evaluating the therapeutic potential of idebenone and related quinone analogues in Leber hereditary optic neuropathy"],"prefix":"10.1016","volume":"36","author":[{"given":"Patrick","family":"Yu-Wai-Man","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Devorah","family":"Soiferman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David G.","family":"Moore","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florence","family":"Burt\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann","family":"Saada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.mito.2017.01.004_bb0005","doi-asserted-by":"crossref","first-page":"377","DOI":"10.4321\/S0365-66912007000600012","article-title":"Response to idebenone and multivitamin therapy in Leber's hereditary optic neuropathy","volume":"82","author":"Barnils","year":"2007","journal-title":"Arch. Soc. Esp. Oftalmol."},{"key":"10.1016\/j.mito.2017.01.004_bb0010","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.preteyeres.2003.10.003","article-title":"Mitochondrial dysfunction as a cause of optic neuropathies","volume":"23","author":"Carelli","year":"2004","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.mito.2017.01.004_bb0015","doi-asserted-by":"crossref","DOI":"10.1093\/brain\/awr180","article-title":"Idebenone treatment in Leber's hereditary optic neuropathy","volume":"134","author":"Carelli","year":"2011","journal-title":"Brain"},{"key":"10.1016\/j.mito.2017.01.004_bb0020","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.brainres.2006.01.068","article-title":"Coenzyme Q(1) depletes NAD(P)H and impairs recycling of ascorbate in astrocytes","volume":"1078","author":"Dragan","year":"2006","journal-title":"Brain Res."},{"key":"10.1016\/j.mito.2017.01.004_bb0025","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1093\/brain\/awq276","article-title":"Oestrogens ameliorate mitochondrial dysfunction in Leber's hereditary optic neuropathy","volume":"134","author":"Giordano","year":"2011","journal-title":"Brain"},{"key":"10.1016\/j.mito.2017.01.004_bb0030","doi-asserted-by":"crossref","DOI":"10.1038\/cddis.2015.364","article-title":"Cigarette toxicity triggers Leber's hereditary optic neuropathy by affecting mtDNA copy number, oxidative phosphorylation and ROS detoxification pathways","volume":"6","author":"Giordano","year":"2015","journal-title":"Cell Death Dis."},{"key":"10.1016\/j.mito.2017.01.004_bb0035","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1016\/j.bbabio.2011.10.012","article-title":"The effects of idebenone on mitochondrial bioenergetics","volume":"1817","author":"Giorgio","year":"2012","journal-title":"Biochim. Biophys. Acta, Bioenerg."},{"key":"10.1016\/j.mito.2017.01.004_bb0040","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0026883","article-title":"Screening for active small molecules in mitochondrial complex I deficient patient's fibroblasts, reveals AICAR as the most beneficial compound","volume":"6","author":"Golubitzky","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.mito.2017.01.004_bb0045","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1002\/ana.24362","article-title":"Prevalence of nuclear and mitochondrial DNA mutations related to adult mitochondrial disease","volume":"77","author":"Gorman","year":"2015","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.mito.2017.01.004_bb0050","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/j.redox.2015.01.009","article-title":"Border between natural product and drug: comparison of the related benzoquinones idebenone and coenzyme Q(10)","volume":"4","author":"Gueyen","year":"2015","journal-title":"Redox Biol."},{"key":"10.1016\/j.mito.2017.01.004_bb0055","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0017963","article-title":"NQO1-dependent redox cycling of idebenone: effects on cellular redox potential and energy levels","volume":"6","author":"Haefeli","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.mito.2017.01.004_bb0060","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.biocel.2014.01.020","article-title":"Coenzyme Q10 as a therapy for mitochondrial disease","volume":"49","author":"Hargreaves","year":"2014","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"10.1016\/j.mito.2017.01.004_bb0065","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0045182","article-title":"Idebenone protects against retinal damage and loss of vision in a mouse model of Leber's hereditary optic neuropathy","volume":"7","author":"Heitz","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.mito.2017.01.004_bb0070","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/0167-4943(89)90014-9","article-title":"Effects of idebenone and related-compounds on respiratory activities of brain mitochondria, and on lipid-peroxidation of their membranes","volume":"8","author":"Imada","year":"1989","journal-title":"Arch. Gerontol. Geriatr."},{"issue":"7","key":"10.1016\/j.mito.2017.01.004_bb0075","doi-asserted-by":"crossref","first-page":"3112","DOI":"10.1167\/iovs.08-3166","article-title":"Quality of life in patients with leber hereditary optic neuropathy","volume":"50","author":"Kirkman","year":"2009","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.mito.2017.01.004_bb0080","doi-asserted-by":"crossref","first-page":"2317","DOI":"10.1093\/brain\/awp158","article-title":"Gene-environment interactions in Leber hereditary optic neuropathy","volume":"132","author":"Kirkman","year":"2009","journal-title":"Brain"},{"key":"10.1016\/j.mito.2017.01.004_bb0085","doi-asserted-by":"crossref","first-page":"2677","DOI":"10.1093\/brain\/awr170","article-title":"A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy","volume":"134","author":"Klopstock","year":"2011","journal-title":"Brain"},{"key":"10.1016\/j.mito.2017.01.004_bb0090","doi-asserted-by":"crossref","DOI":"10.1093\/brain\/aws279","article-title":"Persistence of the treatment effect of idebenone in Leber's hereditary optic neuropathy","volume":"136","author":"Klopstock","year":"2013","journal-title":"Brain"},{"key":"10.1016\/j.mito.2017.01.004_bb0095","doi-asserted-by":"crossref","first-page":"152","DOI":"10.1097\/WNO.0000000000000250","article-title":"Superoxide generation explains common features of optic neuropathies associated with cecocentral scotomas","volume":"35","author":"Levin","year":"2015","journal-title":"J. Neuroophthalmol."},{"key":"10.1016\/j.mito.2017.01.004_bb0100","doi-asserted-by":"crossref","first-page":"20065","DOI":"10.1073\/pnas.1217113109","article-title":"Mouse mtDNA mutant model of Leber hereditary optic neuropathy","volume":"109","author":"Lin","year":"2012","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.mito.2017.01.004_bb0105","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1002\/ana.410420407","article-title":"In vivo skeletal muscle mitochondrial function in Leber's hereditary optic neuropathy assessed by 31P magnetic resonance spectroscopy","volume":"42","author":"Lodi","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.mito.2017.01.004_bb0110","doi-asserted-by":"crossref","first-page":"1894","DOI":"10.1093\/brain\/awm114","article-title":"Novel mutations of ND genes in complex I deficiency associated with mitochondrial encephalopathy","volume":"130","author":"Malfatti","year":"2007","journal-title":"Brain"},{"key":"10.1016\/j.mito.2017.01.004_bb0115","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1136\/jmg.39.3.162","article-title":"Leber hereditary optic neuropathy","volume":"39","author":"Man","year":"2002","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.mito.2017.01.004_bb0120","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1086\/346066","article-title":"The epidemiology of Leber hereditary optic neuropathy in the North East of England","volume":"72","author":"Man","year":"2003","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.mito.2017.01.004_bb0125","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1097\/00041327-200020030-00006","article-title":"Do idebenone and vitamin therapy shorten the time to achieve visual recovery in Leber hereditary optic neuropathy?","volume":"20","author":"Mashima","year":"2000","journal-title":"J. Neuroophthalmol."},{"key":"10.1016\/j.mito.2017.01.004_bb0130","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/0167-4943(89)90008-3","article-title":"Brain distribution of idebenone and its effect on local cerebral glucose-utilization in rats","volume":"8","author":"Nagai","year":"1989","journal-title":"Arch. Gerontol. Geriatr."},{"key":"10.1016\/j.mito.2017.01.004_bb0135","doi-asserted-by":"crossref","first-page":"682","DOI":"10.1016\/j.jcf.2013.04.006","article-title":"Mitochondrial OXPHOS function is unaffected by chronic azithromycin treatment","volume":"12","author":"Negari","year":"2013","journal-title":"J. Cyst. Fibros."},{"key":"10.1016\/j.mito.2017.01.004_bb0140","doi-asserted-by":"crossref","first-page":"2447","DOI":"10.1093\/brain\/awr192","article-title":"Treatment of Leber hereditary optic neuropathy","volume":"134","author":"Newman","year":"2011","journal-title":"Brain"},{"key":"10.1016\/j.mito.2017.01.004_bb0145","doi-asserted-by":"crossref","first-page":"1144","DOI":"10.1038\/sj.eye.6701591","article-title":"Hereditary optic neuropathies","volume":"18","author":"Newman","year":"2004","journal-title":"Eye"},{"key":"10.1016\/j.mito.2017.01.004_bb0150","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/0165-022X(88)90104-2","article-title":"Evaluation of growth rate in adhering cell cultures using a simple colorimetric method","volume":"16","author":"Pelletier","year":"1988","journal-title":"J. Biochem. Biophys. Methods"},{"key":"10.1016\/j.mito.2017.01.004_bb0155","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1038\/nrneurol.2013.129","article-title":"New treatments for mitochondrial disease-no time to drop our standards","volume":"9","author":"Pfeffer","year":"2013","journal-title":"Nat. Rev. Neurol."},{"key":"10.1016\/j.mito.2017.01.004_bb0160","doi-asserted-by":"crossref","first-page":"3693","DOI":"10.1016\/j.bmcl.2011.04.085","article-title":"\u03b1-Tocotrienol quinone modulates oxidative stress response and the biochemistry of aging","volume":"15","author":"Shrader","year":"2011","journal-title":"Bioorg. Med. Chem. Lett."},{"key":"10.1016\/j.mito.2017.01.004_bb0165","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1016\/j.neuroscience.2016.01.042","article-title":"A randomized, placebo-controlled trial of the benzoquinone idebenone in a mouse model of OPA1-related dominant optic atrophy reveals a limited therapeutic effect on retinal ganglion cell dendropathy and visual function","volume":"319","author":"Smith","year":"2016","journal-title":"Neuroscience"},{"key":"10.1016\/j.mito.2017.01.004_bb0170","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/j.biochi.2013.08.024","article-title":"The effect of small molecules on nuclear-encoded translation diseases","volume":"100","author":"Soiferman","year":"2014","journal-title":"Biochimie"},{"key":"10.1016\/j.mito.2017.01.004_bb0175","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.preteyeres.2010.11.002","article-title":"Mitochondrial optic neuropathies - disease mechanisms and therapeutic strategies","volume":"30","author":"Yu-Wai-Man","year":"2011","journal-title":"Prog. Retin. Eye Res."},{"key":"10.1016\/j.mito.2017.01.004_bb0180","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1038\/eye.2014.37","article-title":"Treatment strategies for inherited optic neuropathies: past, present and future","volume":"28","author":"Yu-Wai-Man","year":"2014","journal-title":"Eye"}],"container-title":["Mitochondrion"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1567724917300120?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1567724917300120?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,9,4]],"date-time":"2018-09-04T22:56:23Z","timestamp":1536101783000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1567724917300120"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,9]]},"references-count":36,"alternative-id":["S1567724917300120"],"URL":"https:\/\/doi.org\/10.1016\/j.mito.2017.01.004","relation":{},"ISSN":["1567-7249"],"issn-type":[{"value":"1567-7249","type":"print"}],"subject":[],"published":{"date-parts":[[2017,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Evaluating the therapeutic potential of idebenone and related quinone analogues in Leber hereditary optic neuropathy","name":"articletitle","label":"Article Title"},{"value":"Mitochondrion","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.mito.2017.01.004","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 The Authors. Published by Elsevier B.V.","name":"copyright","label":"Copyright"}]}}