{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,20]],"date-time":"2025-09-20T18:24:22Z","timestamp":1758392662025},"reference-count":29,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1989,10,2]],"date-time":"1989-10-02T00:00:00Z","timestamp":623289600000},"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":[[1990,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p><jats:sc>l<\/jats:sc>\u2010Alloisoleucine formation from<jats:sc>l<\/jats:sc>\u2010isoleucine was studied<jats:italic>in vitro<\/jats:italic> and<jats:italic>in vivo<\/jats:italic>. When a healthy subject was loaded with<jats:sc>l<\/jats:sc>\u2010isoleucine, plasma levels of<jats:sc>l<\/jats:sc>\u2010isoleucine and 3\u2010methyl\u20102\u2010oxopentanoate (KMV), as well as<jats:sc>l<\/jats:sc>\u2010alloisoleucine, increased. Peak values were reached successively and were in the order<jats:sc>l<\/jats:sc>\u2010isoleucine &gt; &gt; KMV &gt; &gt;<jats:sc>l<\/jats:sc>\u2010alloisoleucine. Metabolic clearance of<jats:sc>l<\/jats:sc>\u2010isoleucine and KMV was rapid; clearance of<jats:sc>l<\/jats:sc>\u2010alloisoleucine was considerably delayed. When human skin fibroblast cultures were challenged with<jats:sc>l<\/jats:sc>\u2010isoleucine, KMV accumulated at a gradually decreased rate, whereas<jats:sc>l<\/jats:sc>\u2010alloisoleucine accumulated at a gradually accelerated rate. KMV and<jats:sc>l<\/jats:sc>\u2010alloisoleucine formation were related and depended on the<jats:sc>l<\/jats:sc>\u2010isoleucine concentration applied. In cell lines derived from MSUD patients (classical form), metabolite formation was only about 2\u2010fold higher than in control strains. The relatively small difference between normal and MSUD fibroblasts<jats:italic>in vitro<\/jats:italic> as opposed to the striking differences between healthy subjects and MSUD patients<jats:italic>in vivo<\/jats:italic> are discussed with respect to the significance of physiological mechanisms participating in the formation and degradation of<jats:sc>l<\/jats:sc>\u2010alloisoleucine in man.<\/jats:p>","DOI":"10.1007\/bf01799676","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T13:55:56Z","timestamp":1119362156000},"page":"137-150","source":"Crossref","is-referenced-by-count":20,"title":["On the mechanism of<scp>l<\/scp>\u2010alloisoleucine formation: Studies on a healthy subject and in fibroblasts from normals and patients with maple syrup urine disease"],"prefix":"10.1002","volume":"13","author":[{"given":"P.","family":"Schadewaldt","sequence":"first","affiliation":[{"name":"Institut f\u00fcr Physiologische Chemie II  FRG"},{"name":"Diabetes Forschungsinstitut and  FRG"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H. \u2010W.","family":"Hammen","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Physiologische Chemie II  FRG"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Dalle\u2010Feste","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Physiologische Chemie II  FRG"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"U.","family":"Wendel","sequence":"additional","affiliation":[{"name":"Kinderklinik Universit\u00e4t D\u00fcsseldorf  Moorenstrasse 5 D\u00fcsseldorf D\u20104000 FRG"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1989,10,2]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"227","article-title":"Long\u2010term management of a case of carbamyl phosphate synthetase deficiency using ketoanalogues and hydroxyanalogues of essential amino acids","volume":"58","author":"Batshaw M. 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