| Literature DB >> 6452974 |
R J Truscott, D Malegan, E McCairns, D Burke, L Hick, P Sims, B Halpern, K Tanaka, L Sweetman, W L Nyhan, J Hammond, C Bumack, E A Haan, D M Danks.
Abstract
Two metabolites, 4-hydroxyisovaleric acid and mesaconic acid, have been identified and quantified in the urine of a patient with isovaleric acidemia. These compounds do not appear to have been reported previously as being components of human metabolism. In addition, large quantities of 3-methylbutyrolactone, the lactone of 4-hydroxyisovaleric acid, were observed in the volatile profile obtained by headspace chromatography. The demonstration of 4-hydroxyisovaleric acid supports the contention that urinary methylsuccinic acid seen in patients with isovaleric acidemia has arisen by omega-oxidation of isovaleric acid. The identification of mesaconic acid may indicate that the methylsuccinic acid formed in these patients is subject to further metabolism.Entities:
Mesh：
Substances：
Year: 1981 PMID： 6452974 DOI： 10.1016/0009-8981(81)90348-x
Source DB: PubMed Journal: Clin Chim Acta ISSN： 0009-8981 Impact factor: 3.786