[13C]Valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propinonate as an obligate intermediate.
- 1 September 1975
- journal article
- case report
- Published by Proceedings of the National Academy of Sciences in Proceedings of the National Academy of Sciences
- Vol. 72 (9) , 3692-3696
- https://doi.org/10.1073/pnas.72.9.3692
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.Keywords
This publication has 16 references indexed in Scilit:
- Nuclear magnetic resonance spectroscopy: reinvestigation of carbon-13 spin-lattice relaxation time measurements of amino acids.Proceedings of the National Academy of Sciences, 1975
- Carbon-13 as a Label in Biosynthetic StudiesScience, 1974
- Stable Isotope Tracers in the Life Sciences and MedicineScience, 1973
- Properties of Purified Methylmalonate Semialdehyde Dehydrogenase of Pseudomonas aeruginosaJournal of Biological Chemistry, 1970
- Oxidation of Methylmalonate Semialdehyde to Propionyl Coenzyme A in Pseudomonas aeruginosa Grown on ValineJournal of Biological Chemistry, 1968
- The Metabolism of Propionic AcidPublished by Wiley ,1964
- COENZYME-A THIOL ESTERS OF ISOBUTYRIC, METHACRYLIC, AND BETA-HYDROXYISOBUTYRIC ACIDS AS INTERMEDIATES IN THE ENZYMATIC DEGRADATION OF VALINE1957
- Metabolism of Propionic Acid in Animal TissuesNature, 1955
- Enzymatic synthesis of branched chain acids from amino acids.1955
- ISOTOPE STUDIES ON THE METABOLISM OF VALINEJournal of Biological Chemistry, 1955