{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T20:36:07Z","timestamp":1776890167793,"version":"3.51.2"},"reference-count":35,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2000,12,1]],"date-time":"2000-12-01T00:00:00Z","timestamp":975628800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2000,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We characterized the effect of orthotopic liver transplantation on the catabolism of branched\u2010chain <jats:italic>L<\/jats:italic>\u2010amino acids in a female patient with classical form of maple syrup urine disease. Transplantation was performed at the age of 7.4 years due to a terminal liver failure triggered by a hepatitis A infection. Since then, the patient is on an unrestricted diet and plasma concentrations of branched\u2010chain <jats:italic>L<\/jats:italic>\u2010amino and 2\u2010oxo acids are stable, yet at moderately increased levels (2\u2010 to 3\u2010fold of control). <jats:italic>L<\/jats:italic>\u2010Alloisoleucine concentrations, however, remained remarkably elevated (&gt;5\u2010fold of control). <jats:italic>In vivo<\/jats:italic> catabolism was investigated by measuring the metabolic <jats:italic>L<\/jats:italic>\u2010alloisoleucine clearance and whole\u2010body leucine oxidation in the postabsorptive state. In an oral loading test with 580 \u03bcmol alloisoleucine per kg body wt, the <jats:italic>L<\/jats:italic>\u2010alloisoleucine elimination rate constant (0.067 h<jats:sup>\u22121<\/jats:sup>) was in the normal range (0.069\u00b10.012 h<jats:sup>\u22121<\/jats:sup>, <jats:italic>n<\/jats:italic>=4). In an oral <jats:italic>L<\/jats:italic>\u2010[1\u2010<jats:sup>13<\/jats:sup>C]leucine load (38 \u03bcmol\/kg body wt), 19.5% of the tracer dose applied was recovered in exhaled <jats:sup>13<\/jats:sup>CO<jats:sub>2<\/jats:sub> versus 18.9\u00b13.6% in healthy subjects (<jats:italic>n<\/jats:italic>=10). Thus, the patient exhibited obviously normal whole\u2010body catabolic rates although branched\u2010chain <jats:italic>L<\/jats:italic>\u2010amino acid oxidation was confined to the liver transplant. Most likely, the enhanced substrate supply from extrahepatic sources led to an elevation of the plasma concentrations and thus induced a compensatory enhancement of the metabolic flux through the branched\u2010chain 2\u2010oxo acid dehydrogenase complex in the intact liver tissue.<\/jats:p>","DOI":"10.1023\/a:1026708618507","type":"journal-article","created":{"date-parts":[[2003,11,6]],"date-time":"2003-11-06T18:09:07Z","timestamp":1068142147000},"page":"805-818","source":"Crossref","is-referenced-by-count":31,"title":["Branched\u2010chain L\u2010amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation"],"prefix":"10.1002","volume":"23","author":[{"given":"A.","family":"Bodner\u2010Leidecker","sequence":"first","affiliation":[{"name":"Deutsches Diabetes Forschungsinstitut Heinrich\u2010Heine\u2010Universit\u00e4t  D\u00fcsseldorf Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"U.","family":"Wendel","sequence":"additional","affiliation":[{"name":"Kinderklinik Heinrich\u2010Heine\u2010Universit\u00e4t  D\u00fcsseldorf Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u2010M.","family":"Saudubray","sequence":"additional","affiliation":[{"name":"H\u00f4pital Necker\u2010Enfants\u2010Malades  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Schadewaldt","sequence":"additional","affiliation":[{"name":"Deutsches Diabetes Forschungsinstitut Heinrich\u2010Heine\u2010Universit\u00e4t  D\u00fcsseldorf Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2000,12]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1080\/10256019808036370"},{"issue":"2","key":"e_1_2_1_3_1","first-page":"19","article-title":"Liver transplantation in classical maple syrup urine disease (MSUD): near normalization of branched\u2010chain L\u2010amino acid metabolism","volume":"21","author":"Bodner A","year":"1998","journal-title":"J Inherit Metab Dis"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1152\/jappl.1998.85.5.1744"},{"key":"e_1_2_1_5_1","first-page":"1239","volume-title":"The Metabolic and Mol\u2010ecular Bases of Inherited Disease","author":"Chuang DT","year":"1995"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/254529a0"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.bi.44.070175.004441"},{"key":"e_1_2_1_8_1","first-page":"663","article-title":"Isoenzyme patterns of branched\u2010chain amino acid transaminase in human tissues and tumors","volume":"68","author":"Goto M","year":"1977","journal-title":"Gann"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(78)80601-5"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj3390503"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.1997.272.2.E233"},{"issue":"1","key":"e_1_2_1_12_1","first-page":"37","article-title":"Transplantation for maple syrup urine disease (MSUD) and methylmalonic acidopathy (MMA)","volume":"20","author":"Kaplan P","year":"1997","journal-title":"J Inherit Metab Dis"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI108671"},{"key":"e_1_2_1_14_1","first-page":"363","article-title":"\u00dcber den Gehalt normaler menschlicher Organe an Chlor, Calcium, Magnesium und Eisen sowie an Wasser, Eiwei\u00df und Fett","volume":"24","author":"Magnus\u2010Levy A","year":"1910","journal-title":"Biochem Z"},{"key":"e_1_2_1_15_1","unstructured":"MerineroB Rerez\u2010CerdaC SanzP et al (1994)Liver transplantation (LT) in a spanish MSUD patient. 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