Document Detail

Cis-4-decenoic acid in plasma: a characteristic metabolite in medium-chain acyl-CoA dehydrogenase deficiency.
MedLine Citation:
PMID:  3349606     Owner:  NLM     Status:  MEDLINE    
The profile of organic acids in plasma of patients with a deficiency of medium-chain acyl-CoA dehydrogenase (EC 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.
M Duran; L Bruinvis; D Ketting; J B de Klerk; S K Wadman
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Clinical chemistry     Volume:  34     ISSN:  0009-9147     ISO Abbreviation:  Clin. Chem.     Publication Date:  1988 Mar 
Date Detail:
Created Date:  1988-05-11     Completed Date:  1988-05-11     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9421549     Medline TA:  Clin Chem     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  548-51     Citation Subset:  IM    
University Children's Hospital Het Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands.
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MeSH Terms
Acyl-CoA Dehydrogenase
Acyl-CoA Dehydrogenases / deficiency*
Decanoic Acids / blood
Fatty Acids / blood
Fatty Acids, Monounsaturated / blood*
Fatty Acids, Nonesterified / blood
Octanoic Acids / blood
Sudden Infant Death / blood
Reg. No./Substance:
0/Decanoic Acids; 0/Fatty Acids; 0/Fatty Acids, Monounsaturated; 0/Fatty Acids, Nonesterified; 0/Octanoic Acids; 124-07-2/caprylic acid; 334-48-5/decanoic acid; 505-90-8/4-decenoic acid; EC 1.3.-/Acyl-CoA Dehydrogenases; EC Dehydrogenase

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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