Identification of 3-hydroxy-3-ethylglutaric acid in urine of patients with propionic acidaemia.

Abstract

By gas chromatographic mass spectrometric analysis, 3-hydroxy-3-ethylglutaric acid was identified in the urine of patients with propionyl CoA carboxylase deficiency. Simultaneously, large amounts of 3-hydroxyvaleric acid and 3-oxovaleric acid, as well as a number of metabolites previously well known in this disorder, were also found in the urine of the patients. It is suggested that formation of 3-oxovaleric acid and 3-hydroxyvaleric acid proceeds via 3-hydroxy-3-ethylglutaryl CoA as an intermediate by a mechanism similar to that of ketone body formation.

• Authors: T Kuhara, Y Inoue, T Shinka, I Matsumoto, M Matsuo
• Journal: Biomedical mass spectrometry (Biomed Mass Spectrom) Vol. 10 Issue 12 Pg. 629-32 (Dec 1983) ISSN: 0306-042X [Print] England
• PMID: 6200156 (Publication Type: Journal Article)

Chemical References

• Glutarates
• Propionates
• 3-hydroxy-3-ethylglutaric acid
• Meglutol
• Carboxy-Lyases
• Methylmalonyl-CoA Decarboxylase
• propionic acid

Topics

• Carboxy-Lyases (deficiency)
• Female
• Gas Chromatography-Mass Spectrometry (methods)
• Glutarates (urine)
• Humans
• Infant
• Meglutol (analogs & derivatives, urine)
• Methylmalonyl-CoA Decarboxylase
• Propionates (blood)