{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T22:12:53Z","timestamp":1793052773342,"version":"4.5.0"},"reference-count":38,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2007,11,17]],"date-time":"2007-11-17T00:00:00Z","timestamp":1195257600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Metab Brain Dis"],"published-print":{"date-parts":[[2008,3]]},"DOI":"10.1007\/s11011-007-9077-y","type":"journal-article","created":{"date-parts":[[2007,11,16]],"date-time":"2007-11-16T20:15:16Z","timestamp":1195244116000},"page":"71-80","source":"Crossref","is-referenced-by-count":41,"title":["Oxidative stress in plasma from maple syrup urine disease patients during treatment"],"prefix":"10.1007","volume":"23","author":[{"given":"Aleth\u00e9a G.","family":"Barschak","sequence":"first","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":"Amanda T.","family":"Barden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos S.","family":"Dutra-Filho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moacir","family":"Wajner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen R.","family":"Vargas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2007,11,17]]},"reference":[{"key":"9077_CR1","volume-title":"Practical statistics for medical research","author":"DG Altman","year":"1991","unstructured":"Altman DG (1991) Practical statistics for medical research. Chapman & Hall, London"},{"key":"9077_CR2","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1016\/S0197-0186(00)00100-5","volume":"38","author":"PR Ara\u00fajo","year":"2001","unstructured":"Ara\u00fajo PR, Wassermann GF, Tallini K, Furlanetto V, Vargas CR, Wannmacher CMD, Dutra-Filho CS, Wyse ATS, Wajner M (2001) Reduction of large neutral amino acid level in plasma and brain of hyperleucinemic rats. Neurochem Int 38:529\u2013537","journal-title":"Neurochem Int"},{"key":"9077_CR3","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1007\/s11011-006-9030-5","volume":"21","author":"AG Barschak","year":"2006","unstructured":"Barschak AG, Sitta A, Deon M, Oliveira MH, Haeser A, Dutra-Filho CS, Wajner M, Vargas CR (2006) Evidence that oxidative stress is increased in plasma from patients with Maple Syrup Urine Disease. Metab Brain Dis 21:279\u2013286","journal-title":"Metab Brain Dis"},{"key":"9077_CR4","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1016\/S0920-1211(00)00096-6","volume":"40","author":"E Ben\u2013Menachem","year":"2000","unstructured":"Ben\u2013Menachem E, Kyllerman R, Markleind S (2000) Superoxide dismutase and glutathione peroxidase function in progressive myoclonus epilepsies. Epilepsy Res 40:33\u201339","journal-title":"Epilepsy Res"},{"key":"9077_CR5","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/S0736-5748(03)00074-1","volume":"21","author":"R Bridi","year":"2003","unstructured":"Bridi R, Araldi J, Sgarbi MB, Testa CG, Durigon K, Wajner M, Dutra-Filho CS (2003) Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. Int J Dev Neurosci 21:327\u2013332","journal-title":"Int J Dev Neurosci"},{"key":"9077_CR6","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1007\/s11011-005-4152-8","volume":"20","author":"R Bridi","year":"2005","unstructured":"Bridi R, Braun CA, Zorzi GK, Wannmacher CMD, Wajner M, Lissi EG, Dutra-Filho CS (2005a) Alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats. Metab Brain Dis 20:155\u2013167","journal-title":"Metab Brain Dis"},{"key":"9077_CR7","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1080\/10715760400022350","volume":"39","author":"R Bridi","year":"2005","unstructured":"Bridi R, Latini A, Braum CA, Zorzi GK, Wajner M, Lissi E, Dutra-Filho CS (2005b) Evaluation of the mechanism involved in leucine-induced oxidative damage in cerebral c\u00f3rtex of young rats. Free Radic Res 39:71\u201379","journal-title":"Free Radic Res"},{"key":"9077_CR8","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1016\/S0076-6879(78)52032-6","volume":"52","author":"JA Buege","year":"1978","unstructured":"Buege JA, Aust SD (1978) Microssomal lipid peroxidation. Methods Enzymol 52:302\u2013309","journal-title":"Methods Enzymol"},{"key":"9077_CR9","first-page":"1971","volume-title":"The metabolic and molecular bases of inherited disease","author":"DT Chuang","year":"2001","unstructured":"Chuang DT, Shih VE (2001) Maple syrup urine disease (branched-chain ketoaciduria). In: Scriver CR, Beaudt AL, Sly WL, Valle D (eds) The metabolic and molecular bases of inherited disease. McGraw-Hill, New York, pp 1971\u20132005"},{"key":"9077_CR10","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/S0006-8993(00)03321-7","volume":"894","author":"AS Coitinho","year":"2001","unstructured":"Coitinho AS, de Mello CF, Lima TT, de Bastiani J, Fighera MR, Wajner M (2001) Pharmacological evidence that alpha-keto isovaleric acid induces convulsions through GABAergic and glutamatergic mechanisms in rats. Brain Res 894:68\u201373","journal-title":"Brain Res"},{"key":"9077_CR11","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/S0025-7753(00)71479-5","volume":"115","author":"C Colome","year":"2000","unstructured":"Colome C, Sierra C, Vilaseca MA (2000) Congenital errors of metabolism: cause of oxidative stress? Med Clin 115:111\u2013117","journal-title":"Med Clin"},{"key":"9077_CR12","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/0076-6879(90)86134-H","volume":"186","author":"H Esterbauer","year":"1990","unstructured":"Esterbauer H, Cheeseman KH (1990) Determination of aldehydic lipid peroxidation products: malonaldehyde and 4-hydroxynonenal. Methods Enzymol 186:407\u2013421","journal-title":"Methods Enzymol"},{"key":"9077_CR13","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1023\/A:1014004414733","volume":"17","author":"FU Fontella","year":"2002","unstructured":"Fontella FU, Gassen E, Pulrolnik V, Wannmacher CMD, Klein AB, Wajner M, Dutra CS (2002) Stimulation of lipid peroxidation in vitro in rat brain by metabolites accumulating in maple syrup urine disease. Metab Brain Dis 17:47\u201354","journal-title":"Metab Brain Dis"},{"key":"9077_CR14","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1016\/S0021-9258(18)57021-6","volume":"177","author":"AG Gornall","year":"1949","unstructured":"Gornall AG, Bardawill CJ, David MM (1949) Determination of serum proteins by means of the biuret reaction. J Biol Chem 177:751\u2013766","journal-title":"J Biol Chem"},{"key":"9077_CR15","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1016\/S0140-6736(94)92211-X","volume":"344","author":"B Halliwell","year":"1994","unstructured":"Halliwell B (1994) Free radicals, antioxidants and human disease: curiosity, cause or consequence? Lancet 344:721\u2013724","journal-title":"Lancet"},{"key":"9077_CR16","unstructured":"Halliwell B, Gutteridge JMC (eds) (2001) Free radicals in biology and medicine. Oxford University Press, Oxford"},{"key":"9077_CR17","first-page":"13","volume-title":"HPLC of small peptides","author":"MH Joseph","year":"1986","unstructured":"Joseph MH, Marsden CA (1986) Amino acids and small peptides. In: Lim CF (ed) HPLC of small peptides. IRL Press, Oxford, pp 13\u201327"},{"key":"9077_CR18","doi-asserted-by":"crossref","first-page":"1919","DOI":"10.1091\/mbc.11.5.1919","volume":"11","author":"P Jouvet","year":"2000","unstructured":"Jouvet P, Rustin P, Taylor DL, Pocock JM, Felderhoff-Mueser U, Mazarakis ND, Sarraf C, Joashi U, Koszma M, Greewood K, Edwards AD, Mehmet H (2000) Branched chain amino acids induce apoptosis in neural cells without mitochondrial membrane despolarization or cytochrome c release: implications for neurological impairment associated with maple syrup urine disease. Mol Biol Cell 11:1919\u20131932","journal-title":"Mol Biol Cell"},{"key":"9077_CR19","doi-asserted-by":"crossref","first-page":"299","DOI":"10.3109\/10715769209079523","volume":"17","author":"E Lissi","year":"1992","unstructured":"Lissi E, Pascual C, Del Castillo MD (1992) Luminol luminescence induced by 2,2\u2032-azo-bis-(2-amidinopropane) thermolysis. Free Radic Res Commun 17:299\u2013311","journal-title":"Free Radic Res Commun"},{"key":"9077_CR20","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/0891-5849(94)00117-3","volume":"18","author":"E Lissi","year":"1995","unstructured":"Lissi E, Salim-Hanna M, Pascual C, Del Castillo MD (1995) Evaluation of total antioxidant potential (TRAP) and total antioxidant reactivity from luminol-enhanced chemiluminescence measurements. Free Radic Biol Med 18:153\u2013158","journal-title":"Free Radic Biol Med"},{"key":"9077_CR21","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1042\/cs0840407","volume":"84","author":"NJ Miller","year":"1993","unstructured":"Miller NJ, Rice-Evans C, Davies MJ, Gopinathan V, Milner A (1993) A novel method for measuring antioxidant capacity and its application to monitoring the antioxidant status in premature neonates. Clin Sci 84:407\u2013412","journal-title":"Clin Sci"},{"key":"9077_CR22","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1023\/A:1021974517837","volume":"18","author":"C Pilla","year":"2003","unstructured":"Pilla C, de Oliveira Cardozo RF, Dutra-Filho CS, Wyze ATS, Wajner M, Wannmacher CMD (2003) Effect of leucine administration on creatine kinase activity in rat brain. Metab Brain Dis 18:17\u201325","journal-title":"Metab Brain Dis"},{"key":"9077_CR23","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1002\/ana.410390204","volume":"39","author":"S Przedborski","year":"1996","unstructured":"Przedborski S, Donaldson DBS, Jakowec M, Kish JS, Guttman M, Rosoklija G, Hays AP (1996) Brain superoxide dismutase, catalase and glutathione peroxidase activities in amyotrophic lateral sclerosis. Ann Neurol 39:158\u2013165","journal-title":"Ann Neurol"},{"key":"9077_CR24","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1007\/978-3-0348-9116-5_37","volume-title":"Free radicals: from basic science to medicine","author":"AZ Reznick","year":"1993","unstructured":"Reznick AZ, Packer L (1993) Free radicals and antioxidants in muscular neurological diseases and disorders. In: Poli G, Albano E, Dianzani MU (eds) Free radicals: from basic science to medicine. Birkh\u00e4user Verlag, Basel, pp 425\u2013437"},{"issue":"suppl. 1","key":"9077_CR25","doi-asserted-by":"crossref","first-page":"S62","DOI":"10.1007\/PL00014274","volume":"156","author":"P Schadewaldt","year":"1997","unstructured":"Schadewaldt P, Wendel U (1997) Metabolism of branched-chain amino acids in maple syrup urine disease. Eur J Pediatr 156(suppl. 1):S62\u2013S66","journal-title":"Eur J Pediatr"},{"key":"9077_CR26","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1016\/j.ymgme.2004.01.016","volume":"82","author":"S Sch\u00f6nberger","year":"2004","unstructured":"Sch\u00f6nberger S, Schweiger B, Schwahn B, Schwarz M, Wendel U (2004) Dysmyelination in the brain of adolescents and young adults with maple syrup urine disease. Mol Genet Metab 82:69\u201375","journal-title":"Mol Genet Metab"},{"key":"9077_CR27","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1016\/j.bbadis.2003.09.010","volume":"1639","author":"AM Sgaravatti","year":"2003","unstructured":"Sgaravatti AM, Rosa RB, Schuck PF, Ribeiro CAJ, Wannmacher CMD, Wyse ATS, Dutra-Filho CS, Wajner M (2003) Inhibition of brain energy metabolism by the \u03b1-keto acids accumulating in maple syrup urine disease. Biochim Biophys Acta 1639:232\u2013238","journal-title":"Biochim Biophys Acta"},{"key":"9077_CR28","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/j.bbadis.2005.02.005","volume":"1740","author":"LR Sirtori","year":"2005","unstructured":"Sirtori LR, Dutra-Filho CS, Fitarelli D, Sitta A, Haeser A, Barschak AG, Wajner M, Coelho DM, Llesuy S, Bell\u00f3-Klein A, Giugliani R, Deon M, Vargas CR (2005) Oxidative stress in patient with phenylketonuria. Biochim Biophys Acta 1740:68\u201373","journal-title":"Biochim Biophys Acta"},{"key":"9077_CR29","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1542\/peds.34.4.454","volume":"34","author":"SE Snyderman","year":"1964","unstructured":"Snyderman SE, Norton PM, Roitman E, Holt LE Jr (1964) Maple syrup urine disease with particular reference to dietotherapy. Pediatrics 34:454\u2013472","journal-title":"Pediatrics"},{"key":"9077_CR30","first-page":"109","volume":"53","author":"T Taketomi","year":"1983","unstructured":"Taketomi T, Kunishita T, Hara A, Mizushima S (1983) Abnormal protein and lipid compositions of the cerebral myelin in patient with maple syrup urine disease. Jpn J Exp Med 53:109\u2013116","journal-title":"Jpn J Exp Med"},{"key":"9077_CR31","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/S0022-510X(00)00402-0","volume":"181","author":"RG Tavares","year":"2000","unstructured":"Tavares RG, Santos CE, Tasca CI, Wajner M, Souza DO, Dutra-Filho CS (2000) Inhibition of glutamate uptake into synaptic vesicles of rat brain by the metabolites accumulating in maple syrup urine disease. J Neurol Sci 181:44\u201349","journal-title":"J Neurol Sci"},{"key":"9077_CR32","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1007\/BF01800354","volume":"15","author":"E Treacy","year":"1992","unstructured":"Treacy E, Clow CL, Reade TR, Chitayat D, Mamer OA, Scriver CR (1992) Maple syrup urine disease: interrelationship between branched-chain amino-, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination. J Inherit Metab Dis 15:121\u2013135","journal-title":"J Inherit Metab Dis"},{"key":"9077_CR33","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1016\/0006-291X(83)90799-4","volume":"114","author":"D Tribble","year":"1983","unstructured":"Tribble D, Shapira R (1983) Myelin proteins: degradation in rat brain initiated by metabolites causative of maple syrup urine disease. Biochem Biophys Res Commun 114:440\u2013446","journal-title":"Biochem Biophys Res Commun"},{"key":"9077_CR34","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/j.bbadis.2003.10.004","volume":"1688","author":"CR Vargas","year":"2004","unstructured":"Vargas CR, Wajner M, Sirtori LR, Goulart L, Chiochetta M, Coelho D, Latini A, Llesuy S, Bell\u00f3-Klein A, Giugliani R, Deon M, Mello CF (2004) Evidence that oxidative stress is increased in patients with X-linked adrenoleukodystrophy. Biochim Biophys Acta 1688:26\u201332","journal-title":"Biochim Biophys Acta"},{"key":"9077_CR35","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1023\/A:1005668431926","volume":"23","author":"M Wajner","year":"2000","unstructured":"Wajner M, Coelho DM, Barschak AG, Araujo PR, Pires RF, Lulhier FL, Vargas CR (2000) Reduction of large neutral amino acid concentrations in plasma and CSF of patients with maple syrup urine disease during crises. J Inherit Metab Dis 23:505\u2013512","journal-title":"J Inherit Metab Dis"},{"key":"9077_CR36","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1023\/B:BOLI.0000037353.13085.e2","volume":"27","author":"M Wajner","year":"2004","unstructured":"Wajner M, Latini A, Wyse ATS, Dutra-Filho CS (2004) The role of oxidative damage in the neuropathology of organic acidurias: insights from animal studies. J Inherit Metab Dis 27:427\u2013448","journal-title":"J Inherit Metab Dis"},{"key":"9077_CR37","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1006\/abio.1999.4314","volume":"275","author":"TW Yu","year":"1999","unstructured":"Yu TW, Ong CN (1999) Lag-time measurement of antioxidant capacity using myoglobin and 2,29-azino-bis(3-ethyl-benzthiazoline-6-sulfonic acid): rationale, application and limitation. Anal Biochem 275:217\u2013223","journal-title":"Anal Biochem"},{"key":"9077_CR38","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1159\/000111436","volume":"18","author":"HR Zielke","year":"1996","unstructured":"Zielke HR, Huang Y, Tildon JT, Zielke CL, Baab PJ (1996) Elevation of amino acids in the interstitial space of the rat brain following infusion of large neutral amino and keto acids by microdialysis: leucine infusion. Dev Neurosci 18:420\u2013425","journal-title":"Dev Neurosci"}],"container-title":["Metabolic Brain Disease"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11011-007-9077-y.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s11011-007-9077-y\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s11011-007-9077-y","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,14]],"date-time":"2023-05-14T19:45:20Z","timestamp":1684093520000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s11011-007-9077-y"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,11,17]]},"references-count":38,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2008,3]]}},"alternative-id":["9077"],"URL":"https:\/\/doi.org\/10.1007\/s11011-007-9077-y","relation":{},"ISSN":["0885-7490","1573-7365"],"issn-type":[{"value":"0885-7490","type":"print"},{"value":"1573-7365","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,11,17]]}}}