Source:http://linkedlifedata.com/resource/pubmed/id/10674358
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
2
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pubmed:dateCreated |
2000-3-30
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pubmed:abstractText |
L-alloisoleucine (2S, 3R), a diastereomer of L-isoleucine (2S, 3S), is a normal constituent of human plasma. Considerable amounts accumulate in maple syrup urine disease, in which the branched-chain 2-oxo acid dehydrogenase step is impaired. The mechanism of L-alloisoleucine formation, however, is unclear. We addressed this issue by performing oral L-[1-13C]isoleucine loading (38 micromol/kg body wt, 50% 1-13C) in overnight-fasted healthy subjects (n = 4) and measuring the 3-h kinetics of 13C-label incorporation into L-isoleucine plasma metabolites. Compared with L-isoleucine, the time course of 13C-enrichment in the related 2-oxo acid, S-3-methyl-2-oxopentanoate, was only slightly delayed. Peak values, amounting to 18+/-4 and 17+/-3 mol percent excess, respectively, were reached within 35 and 45 min, respectively. The kinetics of 13C-enrichment in S- and R-3-methyl-2-oxopentanoate enantiomorphs were similar and linearly correlated (p << 0.001). In L-alloisoleucine, however, 13C-label accumulated only gradually and in minor amounts. Our results indicate that R-3-methyl-2-oxopentanoate is an immediate and inevitable byproduct of L-isoleucine transamination and further suggest that alloisoleucine is primarily formed via retransamination of 3-methyl-2-oxopenanoate in vivo.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Feb
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pubmed:issn |
0031-3998
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
47
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
271-7
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:10674358-Adult,
pubmed-meshheading:10674358-Carbon Isotopes,
pubmed-meshheading:10674358-Female,
pubmed-meshheading:10674358-Humans,
pubmed-meshheading:10674358-Isoleucine,
pubmed-meshheading:10674358-Male,
pubmed-meshheading:10674358-Models, Chemical,
pubmed-meshheading:10674358-Reference Values
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pubmed:year |
2000
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pubmed:articleTitle |
Formation of L-alloisoleucine in vivo: an L-[13C]isoleucine study in man.
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pubmed:affiliation |
Diabetes Forschungsinstitut, Heinrich-Heine-Universität Düsseldorf, Germany.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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