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

Clin Chim Acta. 1985 Nov 15;152(3):253-60. doi: 10.1016/0009-8981(85)90100-7.

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.

MeSH terms

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

Substances

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