Octanoylglucuronide excretion in patients with a defective oxidation of medium-chain fatty acids.

Abstract

Octanoyl-beta-D-glucuronide was identified in the urine of five patients with hypoketotic hypoglycemia and dicarboxylic aciduria due to a defective beta-oxidation of medium-chain fatty acids. Two subjects who ingested large amounts of medium-chain triglycerides also excreted large amounts of the glucuronide. The substance was extracted from the urine with ethyl acetate and analyzed by: (1) gas chromatography/mass spectrometry (GC-MS) of the trimethylsilyl derivative and (2) preparative one-dimensional thin-layer chromatography followed by enzymatic hydrolysis with beta-glucuronidase and again GC-MS. A quantitative analysis was performed indirectly by measuring the urinary bound octanoate after the removal of octanoylcarnitine. Octanoylglucuronide represents an additional mechanism for the detoxification of octanoate; its formation may be of help for the maintenance of carnitine homeostasis in patients with medium-chain acyl-CoA dehydrogenase deficiency.

• Authors: M Duran, D Ketting, R van Vossen, T E Beckeringh, L Dorland, L Bruinvis, S K Wadman
• Journal: Clinica chimica acta; international journal of clinical chemistry (Clin Chim Acta) Vol. 152 Issue 3 Pg. 253-60 (Nov 15 1985) ISSN: 0009-8981 [Print] Netherlands
• PMID: 4064333 (Publication Type: Journal Article)

Chemical References

• Caprylates
• Fatty Acids
• octanoylglucuronide
• Acyl-CoA Dehydrogenases
• Acyl-CoA Dehydrogenase
• octanoylcarnitine
• Carnitine

Topics

• Acyl-CoA Dehydrogenase
• Acyl-CoA Dehydrogenases (deficiency)
• Caprylates (urine)
• Carnitine (analogs & derivatives, urine)
• Fatty Acids (metabolism)
• Gas Chromatography-Mass Spectrometry
• Humans
• Hydrogen-Ion Concentration
• Lipid Metabolism, Inborn Errors (enzymology, urine)