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Clin Chim Acta ; 152(3): 253-60, 1985 Nov 15.
Article in English | MEDLINE | ID: mdl-4064333

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.


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