Tracer studies of the interconversion of R- and S-methylmalonic semialdehydes in man
- 15 October 1985
- journal article
- research article
- Published by Portland Press Ltd. in Biochemical Journal
- Vol. 231 (2) , 481-484
- https://doi.org/10.1042/bj2310481
Abstract
Two human subjects were given separate oral doses of sodium [2H6]isobutyrate and [methyl-2H3]thymine and the labelling patterns of urinary metabolites were determined. Ingestion of deuterated isobutyrate resulted in the excretion of 2H5-labelled S-3-hydroxyisobutyric acid, formed on the direct catabolic pathway, and of S- and R-[2H4]-3-hydroxyisobutyric acids, formed by the reduction of S- and R-methylmalonic semialdehydes respectively. Only the R-enantiomer of urinary 3-hydroxyisobutyric acid was labelled by thymine. This labelling pattern indicates a flow from S- to R-methylmalonic semialdehyde, suggesting that the R-enantiomer is the substrate of methylmalonic semialdehyde dehydrogenase.This publication has 11 references indexed in Scilit:
- Excessive excretion of β‐alanine and of 3‐hydroxypropionic,R‐ andS‐3‐aminoisobutyric,R‐ andS‐3‐hydroxyisobutyric andS‐2‐(hydroxymethyl)butyric acids probably due to a defect in the metabolism of the corresponding malonic semialdehydesJournal of Inherited Metabolic Disease, 1984
- Metabolism of malonic semialdehyde in manBiochemical Journal, 1983
- Linear relationship between the R- and S-enantiomers of β-aminoisobutyric acid in human urineClinica Chimica Acta; International Journal of Clinical Chemistry, 1981
- Isolation and identification of S(+)-3-hydroxyisobutyric acid in the urine of rats loaded with isobutyric acidBiochimica et Biophysica Acta (BBA) - General Subjects, 1979
- Metabolism in rats in vivo of isobutyrates labeled with stable isotopes at various positions. Identification of propionate as an obligate intermediate.Journal of Biological Chemistry, 1978
- [13C]Valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propinonate as an obligate intermediate.Proceedings of the National Academy of Sciences, 1975
- Properties of Purified Methylmalonate Semialdehyde Dehydrogenase of Pseudomonas aeruginosaJournal of Biological Chemistry, 1970
- D-beta-aminoisobutyrate:pyruvate aminotransferase in mammalian liver and excretion of beta-aminoisobutyrate by man.1969
- COENZYME-A THIOL ESTERS OF ISOBUTYRIC, METHACRYLIC, AND BETA-HYDROXYISOBUTYRIC ACIDS AS INTERMEDIATES IN THE ENZYMATIC DEGRADATION OF VALINE1957
- THE PURIFICATION AND PROPERTIES OF BETA-HYDROXYISOBUTYRIC DEHYDROGENASE1957