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 ω-oxidation of isovaleric acid. The identification of mesaconic acid may indicate that the methylsuccinic acid formed in these patients is subject to further metabolism.
Original language | English (US) |
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Pages (from-to) | 187-203 |
Number of pages | 17 |
Journal | Clinica Chimica Acta |
Volume | 110 |
Issue number | 2-3 |
DOIs | |
State | Published - Mar 5 1981 |
Externally published | Yes |
ASJC Scopus subject areas
- Biochemistry
- Clinical Biochemistry
- Biochemistry, medical