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Clinical Chemistry 34: 548-551, 1988;
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Clinical Chemistry, Vol 34, 548-551, Copyright © 1988 by American Association for Clinical Chemistry

Cis-4-decenoic acid in plasma: a characteristic metabolite in medium- chain acyl-CoA dehydrogenase deficiency

M Duran, L Bruinvis, D Ketting, JB de Klerk and SK Wadman
University Children's Hospital Het Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands.

The profile of organic acids in plasma of patients with a deficiency of medium-chain acyl-CoA dehydrogenase (EC 1.3.99.3) was determined by gas- liquid chromatography of trimethylsilylated derivatives of the acids isolated by ethyl acetate extraction. All 13 patients had increased concentrations of free octanoate, cis-4-decenoate, and decanoate in their plasma. Cis-4-decenoate, an intermediary metabolite of linoleic acid, is pathognomonic of medium-chain acyl-CoA dehydrogenase deficiency. This metabolite does not accumulate in plasma after oral loading with medium-chain triglycerides, in contrast to octanoate and decanoate. Two postmortem plasma samples from victims of infant sudden- death syndrome had detectable octanoate and decanoate, but cis-4- decenoate could not be detected. The identification of cis-4-decenoate in plasma may be an aid in the diagnosis of an inherited defect in oxidation of medium-chain fatty acids.


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