{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T19:48:15Z","timestamp":1773517695238,"version":"3.50.1"},"reference-count":79,"publisher":"American Chemical Society (ACS)","issue":"6","license":[{"start":{"date-parts":[[2025,4,15]],"date-time":"2025-04-15T00:00:00Z","timestamp":1744675200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2025,4,15]],"date-time":"2025-04-15T00:00:00Z","timestamp":1744675200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2025,4,15]],"date-time":"2025-04-15T00:00:00Z","timestamp":1744675200000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-045"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01HL146442"],"award-info":[{"award-number":["R01HL146442"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01HL148151"],"award-info":[{"award-number":["R01HL148151"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R01HL149714"],"award-info":[{"award-number":["R01HL149714"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","award":["R21HL150032"],"award-info":[{"award-number":["R21HL150032"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Proteome Res."],"published-print":{"date-parts":[[2025,6,6]]},"DOI":"10.1021\/acs.jproteome.5c00166","type":"journal-article","created":{"date-parts":[[2025,4,15]],"date-time":"2025-04-15T13:33:41Z","timestamp":1744724021000},"page":"3088-3104","source":"Crossref","is-referenced-by-count":3,"title":["Advancing the Biochemical Understanding of Maple Syrup Urine Disease and the Impact of Liver Transplantation: A Pilot Study"],"prefix":"10.1021","volume":"24","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1739-9225","authenticated-orcid":true,"given":"Sotirios P.","family":"Fortis","sequence":"first","affiliation":[{"name":"Department of Biochemistry and Molecular Genetics","place":["Aurora, United States"]},{"name":"University of Colorado Anschutz Medical Campus","place":["Aurora, United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5199-8670","authenticated-orcid":true,"given":"Abby L.","family":"Grier","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Genetics","place":["Aurora, United States"]},{"name":"University of Colorado Anschutz Medical Campus","place":["Aurora, United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julie A.","family":"Reisz","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Genetics","place":["Aurora, United States"]},{"name":"University of Colorado Anschutz Medical Campus","place":["Aurora, United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Monika","family":"Dzieciatkowska","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Genetics","place":["Aurora, United States"]},{"name":"University of Colorado Anschutz Medical Campus","place":["Aurora, United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca I.","family":"Cendali","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Genetics","place":["Aurora, United States"]},{"name":"University of Colorado Anschutz Medical Campus","place":["Aurora, United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vienna","family":"Kauffman","sequence":"additional","affiliation":[{"name":"Central Pennsylvania Clinic, A Medical Home for Special Children and Adults","place":["Belleville, United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"D. Holmes","family":"Morton","sequence":"additional","affiliation":[{"name":"Central Pennsylvania Clinic, A Medical Home for Special Children and Adults","place":["Belleville, United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2258-6490","authenticated-orcid":true,"given":"Angelo","family":"D\u2019Alessandro","sequence":"additional","affiliation":[{"name":"Department of Biochemistry and Molecular Genetics","place":["Aurora, United States"]},{"name":"University of Colorado Anschutz Medical Campus","place":["Aurora, United States"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"316","published-online":{"date-parts":[[2025,4,15]]},"reference":[{"key":"ref1\/cit1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.14.5.462"},{"key":"ref2\/cit2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.1.5114.91"},{"key":"ref3\/cit3","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddu123"},{"key":"ref4\/cit4","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-003-0039-3"},{"key":"ref5\/cit5","volume-title":"StatPearls","author":"Hassan S. A.","year":"2024"},{"key":"ref6\/cit6","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuint.2022.105360"},{"key":"ref7\/cit7","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2023022052"},{"key":"ref8\/cit8","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2020.00973"},{"key":"ref9\/cit9","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2009.12.007"},{"key":"ref10\/cit10","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2004.01.016"},{"key":"ref11\/cit11","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1994.63041508.x"},{"key":"ref12\/cit12","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-003-0039-3"},{"key":"ref13\/cit13","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.1999.276.3.E596"},{"key":"ref14\/cit14","doi-asserted-by":"publisher","DOI":"10.1053\/j.semperi.2015.03.002"},{"key":"ref15\/cit15","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2023-064370"},{"key":"ref16\/cit16","doi-asserted-by":"publisher","DOI":"10.1542\/peds.61.2.262"},{"key":"ref17\/cit17","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2020.01.006"},{"key":"ref18\/cit18","doi-asserted-by":"publisher","DOI":"10.1086\/323677"},{"key":"ref19\/cit19","doi-asserted-by":"publisher","DOI":"10.1542\/peds.109.6.999"},{"key":"ref20\/cit20","doi-asserted-by":"publisher","DOI":"10.1016\/s0022-3476(05)81035-2"},{"key":"ref21\/cit21","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-6143.2005.01209.x"},{"key":"ref22\/cit22","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2010.01411.x"},{"key":"ref23\/cit23","doi-asserted-by":"publisher","DOI":"10.3390\/ijms25115720"},{"key":"ref24\/cit24","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgmr.2020.100651"},{"key":"ref25\/cit25","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4939-9236-2_2"},{"key":"ref26\/cit26","doi-asserted-by":"publisher","DOI":"10.1002\/rcm.7834"},{"key":"ref27\/cit27","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4939-9236-2_8"},{"key":"ref28\/cit28","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkab382"},{"key":"ref29\/cit29","doi-asserted-by":"publisher","DOI":"10.1016\/j.cmet.2018.10.013"},{"key":"ref30\/cit30","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2011.06.033"},{"key":"ref31\/cit31","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbapap.2014.06.003"},{"key":"ref32\/cit32","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1007\/978-3-319-46503-6_15","volume-title":"Macromolecular Protein Complexes: Structure and Function","author":"White M. R.","year":"2017"},{"key":"ref33\/cit33","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2202949"},{"key":"ref34\/cit34","doi-asserted-by":"publisher","DOI":"10.1111\/trf.14936"},{"key":"ref35\/cit35","doi-asserted-by":"publisher","DOI":"10.1158\/1541-7786.MCR-20-0883"},{"key":"ref36\/cit36","doi-asserted-by":"publisher","DOI":"10.4161\/cc.8.24.10242"},{"key":"ref37\/cit37","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-018-21256-6"},{"key":"ref38\/cit38","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.147056"},{"key":"ref39\/cit39","doi-asserted-by":"publisher","DOI":"10.2147\/tacg.S125962"},{"key":"ref40\/cit40","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2016-05-714816"},{"key":"ref41\/cit41","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2020.246603"},{"key":"ref42\/cit42","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-019-12739-9"},{"key":"ref43\/cit43","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-017-0083-x"},{"key":"ref44\/cit44","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12662"},{"key":"ref45\/cit45","doi-asserted-by":"publisher","DOI":"10.3390\/nu11061356"},{"key":"ref46\/cit46","doi-asserted-by":"publisher","DOI":"10.3390\/nu12051414"},{"key":"ref47\/cit47","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejmcr.2024.100175"},{"key":"ref48\/cit48","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinbiochem.2007.11.015"},{"key":"ref49\/cit49","doi-asserted-by":"publisher","DOI":"10.1007\/s11011-007-9077-y"},{"key":"ref50\/cit50","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.9706"},{"key":"ref51\/cit51","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbc.2024.107906"},{"key":"ref52\/cit52","doi-asserted-by":"publisher","DOI":"10.1016\/j.metabol.2014.05.003"},{"key":"ref53\/cit53","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2005.07.027"},{"key":"ref54\/cit54","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2016.11.010"},{"key":"ref55\/cit55","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/B978-0-12-802896-4.00009-2","volume-title":"Biomarkers in Inborn Errors of Metabolism","author":"Hubert L.","year":"2017"},{"key":"ref56\/cit56","doi-asserted-by":"publisher","DOI":"10.3390\/metabo9020036"},{"key":"ref57\/cit57","doi-asserted-by":"publisher","DOI":"10.1016\/j.atherosclerosis.2024.118569"},{"key":"ref58\/cit58","doi-asserted-by":"publisher","DOI":"10.1007\/s12551-017-0297-z"},{"key":"ref59\/cit59","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcp.2019.02.018"},{"key":"ref60\/cit60","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4652(199911)181:2<179::AID-JCP1>3.0.CO;2-8"},{"key":"ref61\/cit61","doi-asserted-by":"publisher","DOI":"10.1038\/s44318-024-00117-1"},{"key":"ref62\/cit62","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M513452200"},{"key":"ref63\/cit63","doi-asserted-by":"publisher","DOI":"10.1111\/bph.14479"},{"key":"ref64\/cit64","doi-asserted-by":"publisher","DOI":"10.1016\/j.chembiol.2008.07.018"},{"key":"ref65\/cit65","volume-title":"GeneReviews(\u00ae)","author":"Kaler S. G.","year":"1993"},{"key":"ref66\/cit66","doi-asserted-by":"publisher","DOI":"10.1177\/25152564211001213"},{"key":"ref67\/cit67","doi-asserted-by":"publisher","DOI":"10.1089\/ars.2010.3492"},{"key":"ref68\/cit68","doi-asserted-by":"publisher","DOI":"10.3945\/ajcn.110.001933"},{"key":"ref69\/cit69","doi-asserted-by":"publisher","DOI":"10.1016\/j.biopha.2016.12.024"},{"key":"ref70\/cit70","doi-asserted-by":"publisher","DOI":"10.1038\/sj.cr.7290105"},{"key":"ref71\/cit71","doi-asserted-by":"publisher","DOI":"10.1038\/ja.2017.104"},{"key":"ref72\/cit72","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/18.14.4278"},{"key":"ref73\/cit73","doi-asserted-by":"publisher","DOI":"10.1038\/s41580-019-0133-3"},{"key":"ref74\/cit74","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1391.018"},{"key":"ref75\/cit75","doi-asserted-by":"publisher","DOI":"10.1016\/j.tibs.2016.05.013"},{"key":"ref76\/cit76","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamem.2011.04.006"},{"key":"ref77\/cit77","doi-asserted-by":"publisher","DOI":"10.1046\/j.1538-7836.2003.00063.x"},{"key":"ref78\/cit78","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M107242200"},{"key":"ref79\/cit79","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddq507"}],"container-title":["Journal of Proteome Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pubs.acs.org\/doi\/pdf\/10.1021\/acs.jproteome.5c00166","content-type":"application\/pdf","content-version":"vor","intended-application":"unspecified"},{"URL":"https:\/\/pubs.acs.org\/doi\/pdf\/10.1021\/acs.jproteome.5c00166","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,6,6]],"date-time":"2025-06-06T08:14:25Z","timestamp":1749197665000},"score":1,"resource":{"primary":{"URL":"https:\/\/pubs.acs.org\/doi\/10.1021\/acs.jproteome.5c00166"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4,15]]},"references-count":79,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2025,6,6]]}},"alternative-id":["10.1021\/acs.jproteome.5c00166"],"URL":"https:\/\/doi.org\/10.1021\/acs.jproteome.5c00166","relation":{},"ISSN":["1535-3893","1535-3907"],"issn-type":[{"value":"1535-3893","type":"print"},{"value":"1535-3907","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,4,15]]}}}