Clin Chim Acta. 1992 Jun 15;208(1-2):85-91.

Determination of (S)- and (R)-2-oxo-3-methylvaleric acid in plasma of patients with maple syrup urine disease.

Wendel U, Even G, Langenbeck U, Schadewaldt P, Hummel W.

Source

Kinderklinik, Heinrich-Heine-Universität, Düsseldorf, FRG.

Abstract

An enzymatic method for the separate measurement of both chiral 2-oxo-3-methylvaleric acid (OMV) compounds, (S)- and (R)-OMV, by NADH-dependent enantioselective amination using leucine dehydrogenase in the presence of a NADH regenerating system is described. This method allows the quantitative determination of all branched-chain 2-oxo acids, simultaneously. In plasma samples from classical maple syrup urine disease patients under therapy the average (R)-OMV/(S)-OMV ratio was 0.35 and great differences in the transamination equilibria of the diastereomeric branched-chain amino acids L-isoleucine and L-alloisoleucine were demonstrated.

PMID:: 1638756; [PubMed - indexed for MEDLINE]

Publication Types, MeSH Terms, Substances

Publication Types

Research Support, Non-U.S. Gov't

MeSH Terms

Substances

LinkOut - more resources

Supplemental Content

Save items

Related citations in PubMed

Determination of R- and S-3-methyl-2-oxopentanoate enantiomers in human plasma: suitable method for label enrichment analysis. [J Chromatogr B Biomed Appl. 1996]
Determination of R- and S-3-methyl-2-oxopentanoate enantiomers in human plasma: suitable method for label enrichment analysis.
Schadewaldt P, Wendel U, Hammen HW. J Chromatogr B Biomed Appl. 1996 Jul 12; 682(2):209-18.
Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease. [J Inherit Metab Dis. 1999]
Renal clearance of branched-chain L-amino and 2-oxo acids in maple syrup urine disease.
Schadewaldt P, Hammen HW, Ott AC, Wendel U. J Inherit Metab Dis. 1999 Aug; 22(6):706-22.
On the mechanisms of the formation of L-alloisoleucine and the 2-hydroxy-3-methylvaleric acid stereoisomers from L-isoleucine in maple syrup urine disease patients and in normal humans. [J Biol Chem. 1992]
On the mechanisms of the formation of L-alloisoleucine and the 2-hydroxy-3-methylvaleric acid stereoisomers from L-isoleucine in maple syrup urine disease patients and in normal humans.
Mamer OA, Reimer ML. J Biol Chem. 1992 Nov 5; 267(31):22141-7.
Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease. [Clin Chim Acta. 1978]
Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease.
Langenbeck U, Wendel U, Mench-Hoinowski A, Kuschel D, Becker K, Przyrembel H, Bremer HJ. Clin Chim Acta. 1978 Sep 1; 88(2):283-91.
Review Metabolism of branched-chain amino acids in maple syrup urine disease. [Eur J Pediatr. 1997]
Review Metabolism of branched-chain amino acids in maple syrup urine disease.
Schadewaldt P, Wendel U. Eur J Pediatr. 1997 Aug; 156 Suppl 1:S62-6.

See reviews... See all...

Related information

Related Citations
Calculated set of PubMed citations closely related to the selected article(s) retrieved using a word weight algorithm. Related articles are displayed in ranked order from most to least relevant, with the “linked from” citation displayed first.
Compound
PubChem chemical compound records that cite the current articles. These references are taken from those provided on submitted PubChem chemical substance records. Multiple substance records may contribute to the PubChem compound record.
Compound (MeSH Keyword)
PubChem chemical compound records that are classified under the same Medical Subject Headings (MeSH) controlled vocabulary as the current articles.
Substance
PubChem chemical substance records that cite the current articles. These references are taken from those provided on submitted PubChem chemical substance records.
Substance (MeSH Keyword)
PubChem chemical substance (submitted) records that are classified under the same Medical Subject Headings (MeSH) controlled vocabulary as the current articles.

Recent activity

Clear Turn Off Turn On

Determination of (S)- and (R)-2-oxo-3-methylvaleric acid in plasma of patients w...
Determination of (S)- and (R)-2-oxo-3-methylvaleric acid in plasma of patients with maple syrup urine disease.
Clin Chim Acta. 1992 Jun 15 ;208(1-2):85-91.

PubMed
What is Asian Taenia?
What is Asian Taenia?
Parasitol Int. 2006 ;55 Suppl:S137-41. Epub 2006 Jan 4 .

PubMed
Dose-dense adjuvant chemotherapy for node-positive breast cancer in women 60 yea...
Dose-dense adjuvant chemotherapy for node-positive breast cancer in women 60 years and older: feasibility and tolerability in a subset of patients in a randomized trial.
Crit Rev Oncol Hematol. 2006 May ;58(2):166-75. Epub 2006 Jan 18 .

PubMed
A highly standardized, robust, and cost-effective method for genome-wide transcr...
A highly standardized, robust, and cost-effective method for genome-wide transcriptome analysis of peripheral blood applicable to large-scale clinical trials.
Genomics. 2006 May ;87(5):653-64. Epub 2006 Jan 4 .

PubMed
Arrhythmias in tako-tsubo syndrome--benign or malignant?
Arrhythmias in tako-tsubo syndrome--benign or malignant?
Int J Cardiol. 2007 Jan 2 ;114(1):141-4. Epub 2006 Jan 4 .

PubMed

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

PubMed

Result Filters

Display Settings:

Send to:

Determination of (S)- and (R)-2-oxo-3-methylvaleric acid in plasma of patients with maple syrup urine disease.

Source

Abstract

Publication Types, MeSH Terms, Substances

Publication Types

MeSH Terms

Substances

LinkOut - more resources

Full Text Sources

Other Literature Sources

Medical

Supplemental Content

Save items

Related citations in PubMed

Related information

Recent activity

Simple NCBI Directory

Getting Started

Resources

Popular

Featured

NCBI Information