Document Detail


Fatty acid beta-oxidation in leukocytes from control subjects and medium-chain acyl-CoA dehydrogenase deficient patients.
MedLine Citation:
PMID:  1737072     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In recent years an increasing number of inherited diseases in man have been identified in which there is an impairment in mitochondrial fatty acid oxidation. Diagnosis is usually done by gas-chromatographic analysis of urine, which may give difficulties, since urinary abnormalities may only be present intermittently. We therefore studied whether leukocytes could be used to study mitochondrial beta-oxidation directly. The results described herein show that leukocytes are able to beta-oxidize octanoate and palmitate. Furthermore, clear abnormalities in octanoate beta-oxidation were found in leukocytes from patients with an established deficiency of medium-chain acyl-CoA dehydrogenase, suggesting that measurement of octanoate and palmitate beta-oxidation in leukocytes may contribute to rapid diagnosis of medium-chain acyl-CoA dehydrogenase deficiency and presumably other mitochondrial beta-oxidation disorders.
Authors:
R J Wanders; L Ijlst
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Biochimica et biophysica acta     Volume:  1138     ISSN:  0006-3002     ISO Abbreviation:  Biochim. Biophys. Acta     Publication Date:  1992 Jan 
Date Detail:
Created Date:  1992-03-17     Completed Date:  1992-03-17     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0217513     Medline TA:  Biochim Biophys Acta     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  80-4     Citation Subset:  IM    
Affiliation:
Department of Pediatric Clinical Biochemistry, University Hospital Amsterdam, The Netherlands.
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MeSH Terms
Descriptor/Qualifier:
3-Hydroxybutyric Acid
Acyl-CoA Dehydrogenase
Acyl-CoA Dehydrogenases / deficiency*,  urine
Fatty Acids / metabolism*
Humans
Hydroxybutyrates / metabolism
Leukocytes / enzymology,  metabolism*
Lipid Metabolism, Inborn Errors / diagnosis,  enzymology*
Mitochondria / metabolism
Octanoic Acids / metabolism
Oxidation-Reduction / drug effects
Palmitates / metabolism
Time Factors
Chemical
Reg. No./Substance:
0/Fatty Acids; 0/Hydroxybutyrates; 0/Octanoic Acids; 0/Palmitates; 124-07-2/caprylic acid; 300-85-6/3-Hydroxybutyric 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


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