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Analysis of (S)- and (R)-3-methyl-2-oxopentanoate enantiomorphs in body fluids.
Methods Enzymol
February 2001
Deutsches Diabetes Forschungsinstitut, Klinische Biochemie, Düsseldorf, Germany.
Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease.
J Inherit Metab Dis
August 1999
Diabetes-Forschungsinstitut an der Heinrich-Heine-Universität, Düsseldorf, Germany.
In maple syrup urine disease (MSUD), branched-chain L-amino (BCAA) and 2-oxo acids (BCOA) accumulate in body fluids owing to an inherited deficiency of branched-chain 2-oxo acid dehydrogenase complex activity. In MSUD, little information is available on the significance of urinary disposal of branched-chain compounds. We examined the renal clearance of leucine, valine, isoleucine and alloisoleucine, and their corresponding 2-oxo acids 4-methyl-2-oxopentanoate (KIC), 3-methyl-2-oxobutanoate (KIV), (S)-(S-KMV), and (R)-3-methyl-2-oxopentanoate (R-KMV), using pairs of plasma and urine samples (n = 63) from 10 patients with classical MSUD.
View Article and Find Full Text PDFDetermination of R- and S-3-methyl-2-oxopentanoate enantiomers in human plasma: suitable method for label enrichment analysis.
J Chromatogr B Biomed Appl
July 1996
Diabetes Forschungsinstitut, Heinrich-Heine-Universität, Düsseldorf, Germany.
A sensitive method for the determination of S- and R-3-methyl-2-oxopentanoate enantiomers (KMV, alpha-keto-beta-methylvalerate) in physiological fluids suitable for isotope enrichment analysis is described: after extraction with acid, 2-oxo acids are separated from interfering amino acids by cation-exchange chromatography. Reductive amination of the branched-chain 2-oxo acids by use of L-leucine dehydrogenase yields the corresponding L-amino acids. L-Isoleucine and L-alloisoleucine which are formed from S- and R-3-methyl-2-oxopentanoate, respectively, are then quantified by amino acid analysis.
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