{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T22:12:54Z","timestamp":1793052774794,"version":"4.5.0"},"reference-count":28,"publisher":"Elsevier BV","issue":"4-5","license":[{"start":{"date-parts":[[2008,3,1]],"date-time":"2008-03-01T00:00:00Z","timestamp":1204329600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2008,3,1]],"date-time":"2008-03-01T00:00:00Z","timestamp":1204329600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Clinical Biochemistry"],"published-print":{"date-parts":[[2008,3]]},"DOI":"10.1016\/j.clinbiochem.2007.11.015","type":"journal-article","created":{"date-parts":[[2007,12,6]],"date-time":"2007-12-06T11:36:33Z","timestamp":1196940993000},"page":"317-324","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":29,"title":["Maple syrup urine disease in treated patients: Biochemical and oxidative stress profiles"],"prefix":"10.1016","volume":"41","author":[{"given":"Aleth\u00e9a G.","family":"Barschak","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christiane","family":"Marchesan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Sitta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marion","family":"Deon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Giugliani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moacir","family":"Wajner","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.clinbiochem.2007.11.015_bib1","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"},{"issue":"Suppl. 1","key":"10.1016\/j.clinbiochem.2007.11.015_bib2","doi-asserted-by":"crossref","first-page":"S62","DOI":"10.1007\/PL00014274","article-title":"Metabolism of branched-chain amino acids in maple syrup urine disease","volume":"156","author":"Schadewaldt","year":"1997","journal-title":"Eur J Pediatr"},{"issue":"6","key":"10.1016\/j.clinbiochem.2007.11.015_bib3","doi-asserted-by":"crossref","first-page":"716","DOI":"10.1007\/s10545-006-0276-1","article-title":"Variant maple syrup urine disease (MSUD)\u2014the entire spectrum","volume":"29","author":"Schadewaldt","year":"2006","journal-title":"J Inherit Metab Dis"},{"issue":"1","key":"10.1016\/j.clinbiochem.2007.11.015_bib4","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"},{"issue":"4","key":"10.1016\/j.clinbiochem.2007.11.015_bib5","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 Option Neurol"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib6","article-title":"Free radicals in biology and medicine","author":"Halliwell","year":"2001"},{"issue":"9","key":"10.1016\/j.clinbiochem.2007.11.015_bib7","doi-asserted-by":"crossref","first-page":"2675","DOI":"10.1096\/fasebj.6.9.1612291","article-title":"Regulation of antioxidant enzymes","volume":"6","author":"Harris","year":"1992","journal-title":"Faseb J"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib8","first-page":"163","article-title":"Effect of long term bed rest in man on enzymatic antioxidative defence and lipid peroxidation in erythrocytes","volume":"1","author":"Pawlak","year":"1998","journal-title":"J Gravit Physiol"},{"issue":"2","key":"10.1016\/j.clinbiochem.2007.11.015_bib9","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1152\/physrev.1998.78.2.547","article-title":"The free theory of aging matures","volume":"78","author":"Beckman","year":"1998","journal-title":"Phisiol Rev"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib10","first-page":"111","article-title":"Congenital errors of metabolism: cause of oxidative stress?","volume":"115","author":"Colome","year":"2000","journal-title":"M\u00e9d Clin"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib11","first-page":"541","article-title":"Propionic and l-methylmalonic acids induce oxidative stress in brain of young rats","volume":"11","author":"Fontella","year":"2000","journal-title":"Neuro Report"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib12","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/S0006-8993(02)04118-5","article-title":"Glutaric acid induces oxidative stress in brain of young rats","volume":"964","author":"de Oliveira Marques","year":"2003","journal-title":"Brain Res"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib13","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.clinbiochem.2007.11.015_bib14","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 Devl Neuroscience"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib15","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1023\/B:BOLI.0000037353.13085.e2","article-title":"The role of oxidative damage in the neuropathology of organic acidurias: insights from animal studies","volume":"27","author":"Wajner","year":"2004","journal-title":"J Inherit Metab Dis"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib16","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/j.bbadis.2005.02.005","article-title":"Oxidative stress in patient with phenylketonuria","volume":"1740","author":"Sirtori","year":"2005","journal-title":"Biochim Biophys Acta"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib17","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1007\/s11011-006-9035-0","article-title":"Investigation of oxidative stress parameters in treated phenylketonuric patients","volume":"20","author":"Sitta","year":"2006","journal-title":"Metab Brain Dis"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib18","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/j.bbadis.2003.10.004","article-title":"Evidence that oxidative stress is increased in patients with X-linked adrenoleukodystrophy","volume":"1688","author":"Vargas","year":"2004","journal-title":"Biochim Biophys Acta"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib19","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/j.jns.2006.04.004","article-title":"The effect of Lorenzo's oil on oxidative stress in X-linked adrenoleukodystrophy","volume":"247","author":"Deon","year":"2006","journal-title":"J Neurol Sci"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib20","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":"20","author":"Barschak","year":"2006","journal-title":"Metab Brain Dis"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib21","series-title":"HPLC of Small Peptides","first-page":"13","article-title":"Amino acids and small peptides","author":"Joseph","year":"1986"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib22","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1016\/S0076-6879(78)52032-6","article-title":"Microsomal lipid peroxidation","volume":"52","author":"Buege","year":"1978","journal-title":"Methods Enzymol"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib23","doi-asserted-by":"crossref","first-page":"299","DOI":"10.3109\/10715769209079523","article-title":"Luminol luminescence induced by 2,2\u2032-azo-bis-(2-amidinopropane) thermolysis","volume":"17","author":"Lissi","year":"1992","journal-title":"Free Radic Res Commun"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib24","series-title":"Tietz: Fundamentos de Qu\u00edmica Cl\u00ednica. 4\u00b0edi\u00e7\u00e3o","year":"1998"},{"issue":"2","key":"10.1016\/j.clinbiochem.2007.11.015_bib25","first-page":"153","article-title":"The enzymology of skeletal muscle disorders","volume":"20","author":"Lott","year":"1984","journal-title":"Crit Rev Clin Lab Sci"},{"issue":"2","key":"10.1016\/j.clinbiochem.2007.11.015_bib26","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1002\/hep.20548","article-title":"Serum alanine aminotransferase in skeletal muscle diseases","volume":"41","author":"Nathwani","year":"2005","journal-title":"Hepatology"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib27","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/0076-6879(90)86134-H","article-title":"Determination of aldehydic lipid peroxidation products: malonaldehyde and 4-hydroxynonenal","volume":"186","author":"Esterbauer","year":"1990","journal-title":"Methods Enzymol"},{"key":"10.1016\/j.clinbiochem.2007.11.015_bib28","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/0891-5849(94)00117-3","article-title":"Evaluation of total antioxidant potential (TRAP) and total antioxidant reactivity from luminol-enhanced chemiluminescence measurements","volume":"18","author":"Lissi","year":"1995","journal-title":"Free Radic Biol Med"}],"container-title":["Clinical Biochemistry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0009912007004791?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0009912007004791?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,14]],"date-time":"2025-09-14T15:26:55Z","timestamp":1757863615000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0009912007004791"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,3]]},"references-count":28,"journal-issue":{"issue":"4-5","published-print":{"date-parts":[[2008,3]]}},"alternative-id":["S0009912007004791"],"URL":"https:\/\/doi.org\/10.1016\/j.clinbiochem.2007.11.015","relation":{},"ISSN":["0009-9120"],"issn-type":[{"value":"0009-9120","type":"print"}],"subject":[],"published":{"date-parts":[[2008,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Maple syrup urine disease in treated patients: Biochemical and oxidative stress profiles","name":"articletitle","label":"Article Title"},{"value":"Clinical Biochemistry","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.clinbiochem.2007.11.015","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2007 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}