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Literature DB >> 1059159

[13C]Valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propinonate as an obligate intermediate.

K Tanaka, I M Armitage, H S Ramsdell, Y E Hsia, S R Lipsky, L E Rosenberg.

Abstract

[Alpha-13C]- and [alpha,beta-13C]valine were administered sequentially to a patient with methylmalonicacidemia to clarify the metabolic pathway of valine from methylmalonic acid semialdehyde to methylmalonyl-CoA. Methylmalonic acid was isolated from multiple urine samples, purified, and analyzed by 13C nuclear magnetic resonance spectroscopy. Contrary to the widely accepted view, the results show unequivocally that methylmalonic acid semialdehyde is decarboxylated to propionate before conversion to methylmalonyl CoA.

Entities: Chemical Disease Species

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Year: 1975 PMID： 1059159 PMCID： PMC433063 DOI： 10.1073/pnas.72.9.3692

Source DB: PubMed Journal: Proc Natl Acad Sci U S A ISSN： 0027-8424 Impact factor: 11.205

16 in total

Review 1. THE METABOLISM OF PROPIONIC ACID.

Authors: Y KAZIRO; S OCHOA
Journal: Adv Enzymol Relat Subj Biochem Date: 1964

2. Metabolism of propionic acid in animal tissues.

Authors: M FLAVIN; P J ORTIZ; S OCHOA
Journal: Nature Date: 1955-10-29 Impact factor: 49.962

3. Enzymatic synthesis of branched chain acids from amino acids.

Authors: M J COON
Journal: Fed Proc Date: 1955-09

4. Coenzyme A thiol esters of isobutyric, methacrylic, and beta-hydroxyisobutyric acids as intermediates in the enzymatic degradation of valine.

Authors: B K BACHHAWAT; M J COON; F P KUPIECKI; R NAGLE; W G ROBINSON
Journal: J Biol Chem Date: 1957-01 Impact factor: 5.157

5. Isotope studies on the metabolism of valine.

Authors: D S KINNORY; Y TAKEDA; D M GREENBERG
Journal: J Biol Chem Date: 1955-01 Impact factor: 5.157

6. Nuclear magnetic resonance spectroscopy: reinvestigation of carbon-13 spin-lattice relaxation time measurements of amino acids.

Authors: H Pearson; D Gust; I M Armitage; H Huber; J D Roberts; R E Stark; R R Vold; R L Vold
Journal: Proc Natl Acad Sci U S A Date: 1975-04 Impact factor: 11.205

8. Excessive excretion of beta-alanine and of 3-hydroxypropionic, R- and S-3-aminoisobutyric, R- and S-3-hydroxyisobutyric and S-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes.

Authors: R J Pollitt; A Green; R Smith
Journal: J Inherit Metab Dis Date: 1985 Impact factor: 4.982

8 in total

[13C]Valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propinonate as an obligate intermediate.

Review 1. THE METABOLISM OF PROPIONIC ACID.

2. Metabolism of propionic acid in animal tissues.

3. Enzymatic synthesis of branched chain acids from amino acids.

4. Coenzyme A thiol esters of isobutyric, methacrylic, and beta-hydroxyisobutyric acids as intermediates in the enzymatic degradation of valine.

5. Isotope studies on the metabolism of valine.

6. Nuclear magnetic resonance spectroscopy: reinvestigation of carbon-13 spin-lattice relaxation time measurements of amino acids.

Review 7. Carbon-13 as a label in biosynthetic studies.

8. Properties of purified methylmalonate semialdehyde dehydrogenase of Pseudomonas aeruginosa.

9. Oxidation of methylmalonate semialdehyde to propionyl coenzyme A in Pseudomonas aeruginosa grown on valine.

10. Stable isotope tracers in the life sciences and medicine.

1. Hypermethioninaemia and 3-hydroxyisobutyric aciduria in an apparently healthy baby.

2. Demonstration of a new mammalian isoleucine catabolic pathway yielding an Rseries of metabolites.

Review 3. [Methylmalonic aciduria. Classification, diagnosis and therapy (author's transl)].

4. Tracer studies of the interconversion of R- and S-methylmalonic semialdehydes in man.

5. Metabolism of methylmalonic acid in rats. Is methylmalonyl-coenzyme a racemase deficiency symptomatic in man?

6. Propionyl-CoA carboxylase deficiency with overflow of metabolites of isoleucine catabolism at all levels.

7. The role of enoyl-coa hydratase in the metabolism of isoleucine by Pseudomonas putida.

8. Excessive excretion of beta-alanine and of 3-hydroxypropionic, R- and S-3-aminoisobutyric, R- and S-3-hydroxyisobutyric and S-2-(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydes.