{"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":1793052773312,"version":"4.5.0"},"reference-count":80,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2018,5,8]],"date-time":"2018-05-08T00:00:00Z","timestamp":1525737600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2018,5,8]],"date-time":"2018-05-08T00:00:00Z","timestamp":1525737600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"funder":[{"DOI":"10.13039\/501100003593","name":"Conselho Nacional de Desenvolvimento Cient\u00edfico e Tecnol\u00f3gico","doi-asserted-by":"publisher","award":["402047\/2010\u20109"],"award-info":[{"award-number":["402047\/2010\u20109"]}],"id":[{"id":"10.13039\/501100003593","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005667","name":"Funda\u00e7\u00e3o de Amparo \u00e0 Pesquisa e Inova\u00e7\u00e3o do Estado de Santa Catarina","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005667","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2018,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Maple syrup urine disease (MSUD) is an autosomal recessive inherited disorder that affects branched\u2010chain amino acid (BCAA) catabolism and is associated with acute and chronic brain dysfunction. Recent studies have shown that inflammation may be involved in the neuropathology of MSUD. However, these studies have mainly focused on single or small subsets of proteins or molecules. Here we performed a case\u2010control study, including 12 treated\u2010MSUD patients, in order to investigate the plasmatic biomarkers of inflammation, to help to establish a possible relationship between these biomarkers and the disease. Our results showed that MSUD patients in treatment with restricted protein diets have high levels of pro\u2010inflammatory cytokines [IFN\u2010\u03b3, TNF\u2010\u03b1, IL\u20101\u03b2 and IL\u20106] and cell adhesion molecules [sICAM\u20101 and sVCAM\u20101] compared to the control group. However, no significant alterations were found in the levels of IL\u20102, IL\u20104, IL\u20105, IL\u20107, IL\u20108, and IL\u201010 between healthy controls and MSUD patients. Moreover, we found a positive correlation between number of metabolic crisis and IL\u20101\u03b2 levels and sICAM\u20101 in MSUD patients. In conclusion, our findings in plasma of patients with MSUD suggest that inflammation may play an important role in the pathogenesis of MSUD, although this process is not directly associated with BCAA blood levels. Overall, data reported here are consistent with the working hypothesis that inflammation may be involved in the pathophysiological mechanism underlying the brain damage observed in MSUD patients.<\/jats:p>","DOI":"10.1007\/s10545-018-0188-x","type":"journal-article","created":{"date-parts":[[2018,5,8]],"date-time":"2018-05-08T13:40:52Z","timestamp":1525786852000},"page":"631-640","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Evaluation of plasma biomarkers of inflammation in patients with maple syrup urine disease"],"prefix":"10.1002","volume":"41","author":[{"given":"Giselli","family":"Scaini","sequence":"first","affiliation":[{"name":"Laborat\u00f3rio de Bioenerg\u00e9tica e N\u00facleo de Excel\u00eancia em Neuroci\u00eancias Aplicadas de Santa Catarina (NENASC), Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Ci\u00eancias da Sa\u00fade Universidade do Extremo Sul Catarinense Av. Universit\u00e1ria, 1105 Crici\u00fama SC 88806\u2010000 Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T\u00e1ssia","family":"Tonon","sequence":"additional","affiliation":[{"name":"BRAIN Laboratory (Basic Research and Advanced Investigations in Neurosciences) Hospital de Cl\u00ednicas de Porto Alegre Porto Alegre Brazil"},{"name":"Post Graduation Program in Medicine: Medical Sciences Universidade Federal do Rio Grande do Sul Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina F.","family":"Moura de Souza","sequence":"additional","affiliation":[{"name":"Medical Genetics Service Hospital de Cl\u00ednicas de Porto Alegre Porto Alegre RS Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia F.","family":"Schuck","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Erros Inatos do Metabolismo, Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Ci\u00eancias da Sa\u00fade Universidade do Extremo Sul Catarinense Crici\u00fama SC Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gustavo C.","family":"Ferreira","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Neuroqu\u00edmica, Instituto de Biof\u00edsica Carlos Chagas Universidade Federal do Rio de Janeiro Rio de Janeiro Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u00e3o","family":"Quevedo","sequence":"additional","affiliation":[{"name":"Laboratory of Neurosciences, Graduate Program in Health Sciences, Health Sciences Unit University of Southern Santa Catarina (UNESC) Crici\u00fama SC Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jo\u00e3o Seda","family":"Neto","sequence":"additional","affiliation":[{"name":"Hospital Sirio Libanes S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatiana","family":"Amorim","sequence":"additional","affiliation":[{"name":"Associa\u00e7\u00e3o de Pais e Amigos dos Excepcionais (APAE) Salvador Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr","given":"Jose S.","family":"Camelo","sequence":"additional","affiliation":[{"name":"Pediatrics Department, Ribeir\u00e3o Preto Medical School University of S\u00e3o Paulo Ribeir\u00e3o Preto Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Vitoria Barban","family":"Margutti","sequence":"additional","affiliation":[{"name":"Pediatrics Department, Ribeir\u00e3o Preto Medical School University of S\u00e3o Paulo Ribeir\u00e3o Preto Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafael","family":"Hencke Tresbach","sequence":"additional","affiliation":[{"name":"BRAIN Laboratory (Basic Research and Advanced Investigations in Neurosciences) Hospital de Cl\u00ednicas de Porto Alegre Porto Alegre Brazil"},{"name":"Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Gen\u00e9tica e Biologia Molecular Universidade Federal do Rio Grande do Sul (UFRGS) Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernanda","family":"Sperb\u2010Ludwig","sequence":"additional","affiliation":[{"name":"BRAIN Laboratory (Basic Research and Advanced Investigations in Neurosciences) Hospital de Cl\u00ednicas de Porto Alegre Porto Alegre Brazil"},{"name":"Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Gen\u00e9tica e Biologia Molecular Universidade Federal do Rio Grande do Sul (UFRGS) Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raquel","family":"Boy","sequence":"additional","affiliation":[{"name":"Pediatrics Department, Hospital Universit\u00e1rio Pedro Ernesto Universidade do Estado do Rio de Janeiro Rio de Janeiro Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paula F. V.","family":"de Medeiros","sequence":"additional","affiliation":[{"name":"Unidade Acad\u00eamica de Medicina, Hospital Universit\u00e1rio Alcides Carneiro, Universidade Federal de Campina Grande Campina Grande Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ida Vanessa D.","family":"Schwartz","sequence":"additional","affiliation":[{"name":"Medical Genetics Service Hospital de Cl\u00ednicas de Porto Alegre Porto Alegre RS Brazil"},{"name":"Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Gen\u00e9tica e Biologia Molecular Universidade Federal do Rio Grande do Sul (UFRGS) Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emilio Luiz","family":"Streck","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Bioenerg\u00e9tica e N\u00facleo de Excel\u00eancia em Neuroci\u00eancias Aplicadas de Santa Catarina (NENASC), Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Ci\u00eancias da Sa\u00fade Universidade do Extremo Sul Catarinense Av. Universit\u00e1ria, 1105 Crici\u00fama SC 88806\u2010000 Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,5,8]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-017-0083-x"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.brainres.2010.02.018"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2567.2009.03225.x"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/imm.12233"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0197-0186(00)00100-5"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11011-007-9077-y"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinbiochem.2008.12.005"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0736-5748(03)00074-1"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11011-005-4152-8"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.178.2.851"},{"key":"e_1_2_9_12_1","first-page":"1971","volume-title":"The metabolic and molecular bases of inherited disease","author":"Chuang DT","year":"2001"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.1700810209"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13195-015-0117-2"},{"key":"e_1_2_9_15_1","first-page":"272","article-title":"Maple syrup urine disease: branched\u2010chain keto acid decarboxylation in fibroblasts as measured with amino acids and keto acids","volume":"29","author":"Dancis J","year":"1977","journal-title":"Am J Hum Genet"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrn2297"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2013.02.001"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2004-02-0665"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijdevneu.2015.10.001"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biopsych.2010.06.010"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1159\/000369933"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-008-0804-0"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-007-0579-x"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuint.2006.05.007"},{"key":"e_1_2_9_25_1","first-page":"67","article-title":"Creatine and antioxidant treatment prevent the inhibition of creatine kinase activity and the morphological alterations of C6 glioma cells induced by the branched\u2010chain alpha\u2010keto acids accumulating in maple syrup urine disease","volume":"26","author":"Funchal C","year":"2006","journal-title":"Cell Mol Neurobiol"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1163\/016942410X507948"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biopsych.2009.03.015"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.9187"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgmr.2017.03.006"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jad.2016.09.017"},{"key":"e_1_2_9_31_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00234-003-0955-7"},{"key":"e_1_2_9_32_1","first-page":"13","volume-title":"HPLC of small peptides","author":"Joseph MH","year":"1986"},{"key":"e_1_2_9_33_1","doi-asserted-by":"publisher","DOI":"10.1042\/bst026s341"},{"key":"e_1_2_9_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.2000.tb05604.x"},{"key":"e_1_2_9_35_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.11.5.1919"},{"key":"e_1_2_9_36_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-012-9582-y"},{"key":"e_1_2_9_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.psychres.2006.04.023"},{"key":"e_1_2_9_38_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.1.5114.90"},{"key":"e_1_2_9_39_1","doi-asserted-by":"publisher","DOI":"10.1080\/13854046.2013.816372"},{"key":"e_1_2_9_40_1","first-page":"348","article-title":"Maple syrup disease; isolation and identification of organic acids in the urine","volume":"23","author":"Menkes JH","year":"1959","journal-title":"Pediatrics"},{"key":"e_1_2_9_41_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijdevneu.2012.10.109"},{"key":"e_1_2_9_42_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11011-015-9686-9"},{"key":"e_1_2_9_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mrfmmm.2015.03.008"},{"key":"e_1_2_9_44_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI67217"},{"key":"e_1_2_9_45_1","doi-asserted-by":"publisher","DOI":"10.1093\/gerona\/glu132"},{"key":"e_1_2_9_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbi.2017.01.020"},{"key":"e_1_2_9_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2010.04.007"},{"key":"e_1_2_9_48_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11920-011-0232-0"},{"key":"e_1_2_9_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2004.11.001"},{"key":"e_1_2_9_50_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11064-007-9423-9"},{"key":"e_1_2_9_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.imbio.2013.04.008"},{"key":"e_1_2_9_52_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijdevneu.2015.11.002"},{"key":"e_1_2_9_53_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12035-012-8243-3"},{"key":"e_1_2_9_54_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-012-9549-z"},{"key":"e_1_2_9_55_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12035-013-8447-1"},{"key":"e_1_2_9_56_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11064-015-1541-1"},{"key":"e_1_2_9_57_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12035-016-0116-8"},{"key":"e_1_2_9_58_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10265"},{"key":"e_1_2_9_59_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-3223(99)00206-1"},{"key":"e_1_2_9_60_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12035-015-9548-9"},{"key":"e_1_2_9_61_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2003.09.010"},{"key":"e_1_2_9_62_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2010.01411.x"},{"key":"e_1_2_9_63_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1961.00450160001001"},{"key":"e_1_2_9_64_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-006-0315-y"},{"key":"e_1_2_9_65_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000000665"},{"key":"e_1_2_9_66_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10571-013-0002-0"},{"key":"e_1_2_9_67_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2009.12.007"},{"key":"e_1_2_9_68_1","doi-asserted-by":"publisher","DOI":"10.1042\/CS20130497"},{"key":"e_1_2_9_69_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(00)00402-0"},{"key":"e_1_2_9_70_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01800354"},{"key":"e_1_2_9_71_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbi.2016.03.004"},{"key":"e_1_2_9_72_1","doi-asserted-by":"publisher","DOI":"10.1093\/ije\/26.1.224"},{"key":"e_1_2_9_73_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-9-73"},{"key":"e_1_2_9_74_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.80.6.579"},{"key":"e_1_2_9_75_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005668431926"},{"key":"e_1_2_9_76_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11011-007-9046-5"},{"key":"e_1_2_9_77_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000073620.42047.D7"},{"key":"e_1_2_9_78_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-5728(03)00262-5"},{"key":"e_1_2_9_79_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0197-0186(01)00077-8"},{"key":"e_1_2_9_80_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp024"},{"key":"e_1_2_9_81_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2008.05.020"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s10545-018-0188-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10545-018-0188-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10545-018-0188-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-018-0188-x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T03:34:43Z","timestamp":1694748883000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-018-0188-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,5,8]]},"references-count":80,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2018,7]]}},"alternative-id":["10.1007\/s10545-018-0188-x"],"URL":"https:\/\/doi.org\/10.1007\/s10545-018-0188-x","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,5,8]]}}}