{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,31]],"date-time":"2026-01-31T08:39:51Z","timestamp":1769848791736,"version":"3.49.0"},"reference-count":35,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Clinica Chimica Acta"],"published-print":{"date-parts":[[2026,1]]},"DOI":"10.1016\/j.cca.2025.120755","type":"journal-article","created":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T15:07:27Z","timestamp":1764515247000},"page":"120755","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":0,"special_numbering":"C","title":["Inflammation in maple syrup urine disease"],"prefix":"10.1016","volume":"580","author":[{"given":"Ana Kalise","family":"B\u00f6ttcher","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lu\u00edsa","family":"Tedesco","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bianca Gomes","family":"dos Reis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franciele F\u00e1tima","family":"Lopes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D\u00e9bora","family":"Tonelotto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luiza Rodrigues","family":"Wailer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carmen Regla","family":"Vargas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.cca.2025.120755_bb0005","author":"National Organization for Rare Disorders (NORD)"},{"key":"10.1016\/j.cca.2025.120755_bb0010","series-title":"Bioqu\u00edmica","author":"Voet","year":"2013"},{"key":"10.1016\/j.cca.2025.120755_bb0015","author":"Hassan"},{"key":"10.1016\/j.cca.2025.120755_bb0020","series-title":"Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases","author":"Blau","year":"2014"},{"issue":"5","key":"10.1016\/j.cca.2025.120755_bb0025","doi-asserted-by":"crossref","DOI":"10.1002\/mgg3.1616","article-title":"Molecular basis of various forms of maple syrup urine disease in Chilean patients","volume":"9","author":"Campanholi","year":"2021","journal-title":"Mol. Genet. Genomic Med."},{"key":"10.1016\/j.cca.2025.120755_bb0030","doi-asserted-by":"crossref","DOI":"10.1016\/j.neuint.2022.105360","article-title":"Pathophysiology of maple syrup urine disease: focus on the neurotoxic role of the accumulated branched-chain amino acids and branched-chain \u03b1-keto acids","volume":"157","author":"Amaral","year":"2022","journal-title":"Neurochem. Int."},{"key":"10.1016\/j.cca.2025.120755_bb0035","series-title":"Maple Syrup Urine Disease","author":"Strauss","year":"2006"},{"key":"10.1016\/j.cca.2025.120755_bb0040","doi-asserted-by":"crossref","first-page":"1822","DOI":"10.1038\/s41591-019-0675-0","article-title":"Chronic inflammation in the etiology of disease across the life span","volume":"25","author":"Furman","year":"2019","journal-title":"Nat. Med."},{"key":"10.1016\/j.cca.2025.120755_bb0045","author":"Pahwa"},{"issue":"11","key":"10.1016\/j.cca.2025.120755_bb0050","doi-asserted-by":"crossref","first-page":"968","DOI":"10.1038\/embor.2012.142","article-title":"The inflammation theory of disease. The growing realization that chronic inflammation is crucial in many diseases opens new avenues for treatment","volume":"13","author":"Hunter","year":"2012","journal-title":"EMBO Rep."},{"key":"10.1016\/j.cca.2025.120755_bb0055","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.cca.2025.120755_bb0060","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1007\/s10571-013-0002-0","article-title":"Neurological damage in MSUD: the role of oxidative stress","volume":"34","author":"Sitta","year":"2014","journal-title":"Cell. Mol. Neurobiol."},{"key":"10.1016\/j.cca.2025.120755_bb0065","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.ijdevneu.2015.02.003","article-title":"Urinary biomarkers of oxidative damage in maple syrup urine disease: the L-carnitine role","volume":"42","author":"Guerreiro","year":"2015","journal-title":"Int. J. Dev. Neurosci."},{"key":"10.1016\/j.cca.2025.120755_bb0070","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."},{"key":"10.1016\/j.cca.2025.120755_bb0075","first-page":"43","article-title":"L-carnitine supplementation decreases DNA damage in treated MSUD patients","volume":"775","author":"Mescka","year":"2015","journal-title":"Mutat. Res."},{"key":"10.1016\/j.cca.2025.120755_bb0080","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1002\/glia.20072","article-title":"Evidence that the branched-chain alpha-keto acids accumulating in maple syrup urine disease induce morphological alterations and death in cultured astrocytes from rat cerebral cortex","volume":"48","author":"Funchal","year":"2004","journal-title":"Glia"},{"key":"10.1016\/j.cca.2025.120755_bb0085","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1007\/s11011-022-00969-8","article-title":"Coadministration of tianeptine alters behavioral parameters and levels of neurotrophins in a chronic model of maple syrup urine disease","volume":"37","author":"Morais","year":"2022","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.cca.2025.120755_bb0090","doi-asserted-by":"crossref","first-page":"5709","DOI":"10.1007\/s12035-016-0116-8","article-title":"Serum markers of neurodegeneration in maple syrup urine disease","volume":"54","author":"Scaini","year":"2017","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.cca.2025.120755_bb0095","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1007\/s11011-015-9768-8","article-title":"Intracerebroventricular administration of \u03b1-ketoisocaproic acid decreases brain-derived neurotrophic factor and nerve growth factor levels in brain of young rats","volume":"31","author":"Wisniewski","year":"2016","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.cca.2025.120755_bb0100","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1007\/s12035-013-8618-0","article-title":"Coadministration of branched-chain amino acids and lipopolysaccharide causes matrix metalloproteinase activation and blood-brain barrier breakdown","volume":"50","author":"Scaini","year":"2014","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.cca.2025.120755_bb0105","first-page":"650","article-title":"Branched-chain amino acids influence the immune properties of microglial cells and their responsiveness to pro-inflammatory signals","volume":"2013","author":"De Simone","year":"1832","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.cca.2025.120755_bb0110","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1016\/j.freeradbiomed.2017.01.009","article-title":"High concentration of branched-chain amino acids promotes oxidative stress, inflammation and migration of human peripheral blood mononuclear cells via mTORC1 activation","volume":"104","author":"Zhenyukh","year":"2017","journal-title":"Free Radic. Biol. Med."},{"key":"10.1016\/j.cca.2025.120755_bb0115","doi-asserted-by":"crossref","first-page":"3714","DOI":"10.1007\/s12035-015-9313-0","article-title":"Cerebral oedema, blood-brain barrier breakdown and the decrease in Na(+),K(+)-ATPase activity in the cerebral cortex and hippocampus are prevented by dexamethasone in an animal model of maple syrup urine disease","volume":"53","author":"Rosa","year":"2016","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.cca.2025.120755_bb0120","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1016\/j.ijdevneu.2019.07.007","article-title":"Administration of branched-chain amino acids increases the susceptibility to lipopolysaccharide-induced inflammation in young Wistar rats","volume":"78","author":"Wessler","year":"2019","journal-title":"Int. J. Dev. Neurosci."},{"key":"10.1016\/j.cca.2025.120755_bb0125","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.ijdevneu.2015.11.002","article-title":"Administration of branched-chain amino acids alters the balance between pro-inflammatory and anti-inflammatory cytokines","volume":"48","author":"Rosa","year":"2016","journal-title":"Int. J. Dev. Neurosci."},{"issue":"480","key":"10.1016\/j.cca.2025.120755_bb0130","article-title":"Donepezil treatment mitigates cholinergic system alterations, oxidative stress, neuroinflammation and memory impairment induced by branched-chain amino acid administration in rats","volume":"26","author":"Lemos","year":"2024","journal-title":"Behav. Brain Res."},{"key":"10.1016\/j.cca.2025.120755_bb0135","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1007\/s11064-023-04072-x","article-title":"Memantine improves memory and neurochemical damage in a model of maple syrup urine disease","volume":"49","author":"Lemos","year":"2024","journal-title":"Neurochem. Res."},{"key":"10.1016\/j.cca.2025.120755_bb0140","doi-asserted-by":"crossref","first-page":"1573","DOI":"10.1007\/s11011-023-01193-8","article-title":"Acute effects of intracerebroventricular administration of \u03b1-ketoisocaproic acid in young rats on inflammatory parameters","volume":"38","author":"Rabelo","year":"2023","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.cca.2025.120755_bb0145","doi-asserted-by":"crossref","first-page":"1015","DOI":"10.1007\/s11011-021-00686-8","article-title":"Preliminary results of PBA-loaded nanoparticles development and the effect on oxidative stress and neuroinflammation in rats submitted to a chemically induced chronic model of MSUD","volume":"36","author":"Mescka","year":"2021","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.cca.2025.120755_bb0150","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1002\/jdn.10035","article-title":"Ibuprofen during gestation prevents some changes in physical and reflex development in offspring in a model of hyperleucinemia and maternal inflammation","volume":"80","author":"de Franceschi","year":"2020","journal-title":"Int. J. Dev. Neurosci."},{"key":"10.1016\/j.cca.2025.120755_bb0155","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.1007\/s11011-015-9686-9","article-title":"Investigation of inflammatory profile in MSUD patients: benefit of L-carnitine supplementation","volume":"30","author":"Mescka","year":"2015","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.cca.2025.120755_bb0160","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1007\/s10545-018-0188-x","article-title":"Evaluation of plasma biomarkers of inflammation in patients with maple syrupurine disease","volume":"41","author":"Scaini","year":"2018","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.cca.2025.120755_bb0165","first-page":"S120","article-title":"Maple syrup urine disease as a neurodegenerative disease: pathophysiological mechanisms","volume":"39","author":"Streck","year":"2016","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.cca.2025.120755_bb0170","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1186\/1750-1172-9-73","article-title":"Brain\u2013blood amino acid correlates following protein restriction in murine maple syrup urine disease","volume":"9","author":"Vogel","year":"2014","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1016\/j.cca.2025.120755_bb0175","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1172\/JCI67217","article-title":"Biochemical correlates of neuropsychiatric illness in maple syrup urine disease","volume":"123","author":"Muelly","year":"2013","journal-title":"J. Clin. Invest."}],"container-title":["Clinica Chimica Acta"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0009898125006345?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0009898125006345?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,1,30]],"date-time":"2026-01-30T18:00:14Z","timestamp":1769796014000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0009898125006345"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1]]},"references-count":35,"alternative-id":["S0009898125006345"],"URL":"https:\/\/doi.org\/10.1016\/j.cca.2025.120755","relation":{},"ISSN":["0009-8981"],"issn-type":[{"value":"0009-8981","type":"print"}],"subject":[],"published":{"date-parts":[[2026,1]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Inflammation in maple syrup urine disease","name":"articletitle","label":"Article Title"},{"value":"Clinica Chimica Acta","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.cca.2025.120755","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 Elsevier B.V. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}],"article-number":"120755"}}