Capillary gas chromatographic/mass spectrometric analysis of abnormal metabolites in hypoglycin-treated rat urine
- 1 July 1984
- journal article
- research article
- Published by Wiley in Journal of Mass Spectrometry
- Vol. 11 (7) , 332-339
- https://doi.org/10.1002/bms.1200110704
Abstract
Numerous abnormal metabolites were identified in large amounts in the urine of hypoglycin‐treated rats using capillary gas chromatography/mass spectrometry‐computer analysis. These metabolites are not detectable in significant amounts in normal rats' urine. Ten of them have not been previously associated with hypoglycin administration: these are several hydroxy compounds, including those from the valine and isoleucine pathways, 2‐oxo‐adipic acid, n‐butyrylglycine and isovaleryl glucuronide. These results indicate that the pathways of isoleucine and valine netabolism are inhibited at their respective acyl‐CoA dehydrogenation steps, as is the case for fatty acid, leucine and lysine metabolism, as previously shown. The mass spectra of the trimethylsilyl derivatives of cis, cis‐4,7‐decadiene‐1,10‐dioic, cis‐4‐decene‐1,10‐dioic, cis‐4‐octene‐1,8‐dioic acids, and (methylenecyclopropyl)acetylglycine, which were previously identified using nuclear magnetic resonance and oxidative cleavage or acid hydrolysis, are presented for the first time.Keywords
This publication has 13 references indexed in Scilit:
- Specific glutaryl-CoA dehydrogenating activity is deficient in cultured fibroblasts from glutaric aciduria patients.Journal of Clinical Investigation, 1984
- New metabolites in isovaleric acidemiaClinica Chimica Acta; International Journal of Clinical Chemistry, 1981
- Demonstration of a specific mitochondrial isovaleryl-CoA dehydrogenase deficiency in fibroblasts from patients with isovaleric acidemia.Proceedings of the National Academy of Sciences, 1980
- Inhibition of linoleic acid degradation by hypoglycin AFEBS Letters, 1978
- Jamaican Vomiting SicknessNew England Journal of Medicine, 1976
- Selective inhibition of acyl-CoA dehydrogenases by a metabolite of hypoglycinBiochimica et Biophysica Acta (BBA) - Enzymology, 1976
- Isovaleric and α-Methylbutyric Acidemias Induced by Hypoglycin A: Mechanism of Jamaican Vomiting SicknessScience, 1972
- Identification of β-hydroxyisovaleric acid in the urine of a patient with isovaleric acidemiaBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1968
- Methylenecyclopropaneacetic acid, a metabolite of hypoglycinBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1966
- Isovaleric acidemia: a new genetic defect of leucine metabolism.Proceedings of the National Academy of Sciences, 1966