{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T10:48:44Z","timestamp":1773053324968,"version":"3.50.1"},"reference-count":38,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[1999,8,1]],"date-time":"1999-08-01T00:00:00Z","timestamp":933465600000},"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":[[1999,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>In maple syrup urine disease (MSUD), branched\u2010chain L\u2010amino (BCAA) and 2\u2010oxo acids (BCOA) accumulate in body fluids owing to an inherited deficiency of branched\u2010chain 2\u2010oxo acid dehydrogenase complex activity. In MSUD, little information is available on the significance of urinary disposal of branched\u2010chain compounds. We examined the renal clearance of leucine, valine, isoleucine and alloisoleucine, and their corresponding 2\u2010oxo acids 4\u2010methyl\u20102\u2010oxopentanoate (KIC), 3\u2010methyl\u20102\u2010oxobutanoate (KIV), (S)\u2010 (S\u2010KMV), and (R)\u20103\u2010methyl\u20102\u2010oxopentanoate (R\u2010KMV), using pairs of plasma and urine samples (n=63) from 10 patients with classical MSUD. The fractional renal excretion of free BCAA was in the normal range (&lt;0.5%) and indepen\u2010dent of the plasma concentrations. The excretion of bound (N\u2010acylated) BCAA was normal and not significantly dependent on the BCAA plasma concentrations. The fractional renal excretion of BCOA was in the order KIC\u226aKIV&lt;R\u2010KMV\u2264S\u2010KMV (range (%): KIC 0.1\u201325; KIV 0.14\u201321.3; S\u2010KMV 0.26\u201324.6; R\u2010KMV 0.1\u201335.9), significantly correlated with the KIC plasma concentrations, and generally higher than that of the related BCAA. The results show that the renal excretion of free BCAA as well as of the acylated derivatives is negligible. The renal excretion of BCOA, however, to some extent counteracts increases in BCAA concentrations and thus contributes to the lowering of total BCAA pools in MSUD.<\/jats:p>","DOI":"10.1023\/a:1005540016376","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T22:31:09Z","timestamp":1040509869000},"page":"706-722","source":"Crossref","is-referenced-by-count":17,"title":["Renal clearance of branched\u2010chain L\u2010amino and 2\u2010oxo acids in maple syrup urine disease"],"prefix":"10.1002","volume":"22","author":[{"given":"P.","family":"Schadewaldt","sequence":"first","affiliation":[{"name":"Diabetes\u2010Forschungsinstitut an der Heinrich\u2010Heine\u2010Universit\u00e4t  D\u00fcsseldorf Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.\u2010W.","family":"Hammen>","sequence":"additional","affiliation":[{"name":"Diabetes\u2010Forschungsinstitut an der Heinrich\u2010Heine\u2010Universit\u00e4t  D\u00fcsseldorf 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