{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T11:59:59Z","timestamp":1791892799657,"version":"4.3.4"},"reference-count":33,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2014,9,1]],"date-time":"2014-09-01T00:00:00Z","timestamp":1409529600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Gene"],"published-print":{"date-parts":[[2014,9]]},"DOI":"10.1016\/j.gene.2014.07.051","type":"journal-article","created":{"date-parts":[[2014,7,18]],"date-time":"2014-07-18T22:00:44Z","timestamp":1405720844000},"page":"294-298","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":23,"title":["Prevention of DNA damage by l-carnitine induced by metabolites accumulated in maple syrup urine disease in human peripheral leukocytes in vitro"],"prefix":"10.1016","volume":"548","author":[{"given":"Caroline Paula","family":"Mescka","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos Alberto Yasin","family":"Wayhs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilian","family":"Guerreiro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanusa","family":"Manfredini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos Severo","family":"Dutra-Filho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen Regla","family":"Vargas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.gene.2014.07.051_bb0005","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/j.brainres.2010.02.018","article-title":"Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain","volume":"1324","author":"Amaral","year":"2010","journal-title":"Brain Res."},{"key":"10.1016\/j.gene.2014.07.051_bb0010","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1016\/S0197-0186(00)00100-5","article-title":"Reduction of large neutral amino acid level in plasma and brain of hyperleucinemic rats","volume":"38","author":"Ara\u00fajo","year":"2001","journal-title":"Neurochem. Int."},{"key":"10.1016\/j.gene.2014.07.051_bb0015","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1007\/s11011-006-9030-5","article-title":"Evidence that oxidative stress is increased in plasma from patients with Maple Syrup Urine Disease","volume":"21","author":"Barschak","year":"2006","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.gene.2014.07.051_bb0020","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.ijdevneu.2007.05.004","article-title":"Erythrocyte glutathione peroxidase activity and plasma selenium concentration are reduced in maple syrup urine disease patients during treatment","volume":"25","author":"Barschak","year":"2007","journal-title":"Int. J. Dev. Neurosci."},{"key":"10.1016\/j.gene.2014.07.051_bb0025","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1007\/s11011-007-9077-y","article-title":"Oxidative stress in plasma from maple syrup urine disease patients during treatment","volume":"23","author":"Barschak","year":"2008","journal-title":"Metab. Brain Dis."},{"issue":"10","key":"10.1016\/j.gene.2014.07.051_bb0030","doi-asserted-by":"crossref","first-page":"2131","DOI":"10.1093\/carcin\/14.10.2131","article-title":"The effect of l-carnitine and acetyl l-carnitine on the disappearance of DNA single-strand breaks in human peripheral blood lymphocytes","volume":"14","author":"Boerighter","year":"1993","journal-title":"Carcinogenesis"},{"key":"10.1016\/j.gene.2014.07.051_bb0035","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/S0736-5748(03)00074-1","article-title":"Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease","volume":"21","author":"Bridi","year":"2003","journal-title":"Int. J. Dev. Neurosci."},{"key":"10.1016\/j.gene.2014.07.051_bb0040","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1007\/s11011-005-4152-8","article-title":"Alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats","volume":"20","author":"Bridi","year":"2005","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.gene.2014.07.051_bb0045","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1080\/10715760400022350","article-title":"Evaluation of the mechanism involved in leucine-induced oxidative damage in cerebral cortex of young rats","volume":"39","author":"Bridi","year":"2005","journal-title":"Free Radic. Res."},{"key":"10.1016\/j.gene.2014.07.051_bb0050","series-title":"The Metabolic and Molecular Bases of Inherited Disease","first-page":"1971","article-title":"Maple syrup urine disease (branched-chain ketoaciduria)","author":"Chuang","year":"2001"},{"key":"10.1016\/j.gene.2014.07.051_bb0055","first-page":"595","article-title":"l-Carnitine protects gastric mucosa by decreasing ischemia\u2013reperfusion induced lipid peroxidation","volume":"55","author":"Derin","year":"2004","journal-title":"J. Physiol. Pharmacol."},{"key":"10.1016\/j.gene.2014.07.051_bb0060","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1023\/A:1014004414733","article-title":"Stimulation of lipid peroxidation in vitro in rat brain by metabolites accumulating in maple syrup urine disease","volume":"17","author":"Fontella","year":"2002","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.gene.2014.07.051_bb0065","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1016\/j.lfs.2005.05.103","article-title":"Antioxidant and antiradical activities of l-carnitine","volume":"78","author":"Gulcin","year":"2006","journal-title":"Life Sci."},{"key":"10.1016\/j.gene.2014.07.051_bb0070","series-title":"Free Radicals in Biology and Medicine, 4\u00aaed","author":"Halliwell","year":"2007"},{"key":"10.1016\/j.gene.2014.07.051_bb0075","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1007\/s10522-004-2575-y","article-title":"Age dependent alterations in mitochondrial enzymes in cortex, striatum and hippocampus of rat brain\u2014potential role of l carnitine","volume":"5","author":"Haripriya","year":"2004","journal-title":"Biogerontology"},{"key":"10.1016\/j.gene.2014.07.051_bb0080","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1016\/j.ymeth.2009.02.016","article-title":"The comet assay: a sensitive method for detecting DNA damage in individual cells","volume":"48","author":"Liao","year":"2009","journal-title":"Methods"},{"key":"10.1016\/j.gene.2014.07.051_bb0090","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s11011-011-9238-x","article-title":"In vivo neuroprotective effect of l-carnitine against oxidative stress in maple syrup urine disease","volume":"26","author":"Mescka","year":"2011","journal-title":"Metab. Brain Dis."},{"issue":"1","key":"10.1016\/j.gene.2014.07.051_bb0095","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/j.ijdevneu.2012.10.109","article-title":"Protein and lipid damage in maple syrup urine disease patients: l-carnitine effect","volume":"31","author":"Mescka","year":"2013","journal-title":"Int. J. Dev. Neurosci."},{"key":"10.1016\/j.gene.2014.07.051_bb0100","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1016\/j.cca.2005.12.010","article-title":"Oxidative stress-mediated macromolecular damage and dwindle in antioxidant status in aged rat brain regions: role of l-carnitine and dl-alpha-lipoic acid","volume":"368","author":"Muthuswamy","year":"2006","journal-title":"Clin. Chim. Acta"},{"key":"10.1016\/j.gene.2014.07.051_bb0105","doi-asserted-by":"crossref","first-page":"1183","DOI":"10.1177\/002215540104900912","article-title":"A silver staining method for single-cell gel assay","volume":"49","author":"Nadin","year":"2001","journal-title":"J. Histochem. Cytochem."},{"key":"10.1016\/j.gene.2014.07.051_bb0110","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.ijdevneu.2010.01.002","article-title":"Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of l-carnitine supplementation","volume":"28","author":"Ribas","year":"2010","journal-title":"Int. J. Dev. Neurosci."},{"key":"10.1016\/j.gene.2014.07.051_bb0115","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/j.mrgentox.2010.07.008","article-title":"Prevention by l-carnitine of DNA damage induced by propionic and l-methylmalonic acids in human peripheral leukocytes in vitro","volume":"702","author":"Ribas","year":"2010","journal-title":"Mutat. Res."},{"key":"10.1016\/j.gene.2014.07.051_bb0120","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/j.ymgme.2012.04.009","article-title":"DNA damage in an animal model of maple syrup urine disease","volume":"106","author":"Scaini","year":"2012","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.gene.2014.07.051_bb0135","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1016\/j.ymgme.2004.01.016","article-title":"Dysmyelination in the brain of adolescents and young adults with maple syrup urine disease","volume":"82","author":"Sch\u00f6nberger","year":"2004","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.gene.2014.07.051_bb0140","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/0014-4827(88)90265-0","article-title":"A simple technique for quantification of low levels of DNA damage in individuals cells","volume":"175","author":"Singh","year":"1988","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.gene.2014.07.051_bb0145","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1007\/s10571-010-9636-3","article-title":"Evidence that l-carnitine and selenium supplementation reduces oxidative stress in phenylketonuric patients","volume":"31","author":"Sitta","year":"2011","journal-title":"Cell. Mol. Neurobiol."},{"key":"10.1016\/j.gene.2014.07.051_bb0130","article-title":"Neurological damage in MSUD: the role of oxidative stress","volume":"13","author":"Sitta","year":"2013","journal-title":"Cell. Mol. Neurobiol."},{"key":"10.1016\/j.gene.2014.07.051_bb0155","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1093\/mutage\/10.6.555","article-title":"The contribution of excision repair to the DNA effects seen in the alkaline single cell gel test (comet assay)","volume":"10","author":"Speit","year":"1995","journal-title":"Mutagenesis"},{"key":"10.1016\/j.gene.2014.07.051_bb0160","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1007\/s11940-003-0039-3","article-title":"Branched-chain ketoacyl dehydrogenase deficiency: maple syrup disease","volume":"5","author":"Strauss","year":"2003","journal-title":"Curr. Treat. Options Neurol."},{"key":"10.1016\/j.gene.2014.07.051_bb0165","series-title":"Gene Reviews","article-title":"Maple syrup urine disease","author":"Strauss","year":"2006"},{"key":"10.1016\/j.gene.2014.07.051_bb0170","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/S0022-510X(00)00402-0","article-title":"Inhibition of glutamate uptake into synaptic vesicles of rat brain by the metabolites accumulating in maple syrup urine disease","volume":"181","author":"Tavares","year":"2000","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.gene.2014.07.051_bb0175","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1002\/(SICI)1098-2280(2000)35:3<206::AID-EM8>3.0.CO;2-J","article-title":"Single cell gel\/comet assay: guidelines for in vitro and in vivo genetic toxicology testing","volume":"35","author":"Tice","year":"2000","journal-title":"Environ. Mol. Mutagen."},{"key":"10.1016\/j.gene.2014.07.051_bb0180","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1007\/BF01800354","article-title":"Maple syrup urine disease: interrelationship between branched-chain amino-, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination","volume":"15","author":"Treacy","year":"1992","journal-title":"J. Inherit. Metab. Dis."}],"container-title":["Gene"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0378111914008555?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0378111914008555?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,10,1]],"date-time":"2018-10-01T13:50:01Z","timestamp":1538401801000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0378111914008555"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,9]]},"references-count":33,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2014,9]]}},"alternative-id":["S0378111914008555"],"URL":"https:\/\/doi.org\/10.1016\/j.gene.2014.07.051","relation":{},"ISSN":["0378-1119"],"issn-type":[{"value":"0378-1119","type":"print"}],"subject":[],"published":{"date-parts":[[2014,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Prevention of DNA damage by l-carnitine induced by metabolites accumulated in maple syrup urine disease in human peripheral leukocytes in vitro","name":"articletitle","label":"Article Title"},{"value":"Gene","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.gene.2014.07.051","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2014 Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}