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    
Abstract/OtherAbstract:
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.
Authors:
M Duran; L Bruinvis; D Ketting; J B de Klerk; S K Wadman
Related Documents :
1030166 - Antepartum glucocorticoid treatment: the effect of prednisolone on umbilical blood gluc...
9041816 - Usher's syndrome type ic: clinical studies and fine-mapping the disease locus.
6813436 - Plasma free amino acid levels in uremic rats given high and low protein diets or intrav...
15273286 - Red cell membrane and plasma linoleic acid nitration products: synthesis, clinical iden...
15932296 - Visual enantiomeric recognition of amino acid derivatives in protic solvents.
7821776 - Characterization of crustacean hyperglycemic hormone from the crayfish (procambarus cla...
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    
Affiliation:
University Children's Hospital Het Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
Acyl-CoA Dehydrogenase
Acyl-CoA Dehydrogenases / deficiency*
Decanoic Acids / blood
Fatty Acids / blood
Fatty Acids, Monounsaturated / blood*
Fatty Acids, Nonesterified / blood
Humans
Infant
Octanoic Acids / blood
Sudden Infant Death / blood
Chemical
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 1.3.99.3/Acyl-CoA Dehydrogenase

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


Previous Document:  Effect of creatine kinase-MM subtype composition on a CK-MB immunoinhibition assay.
Next Document:  Excretion of digoxin-like immunoreactivity in urine of normal subjects: correlations with excretion ...