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    
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.
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    
Department of Pediatric Clinical Biochemistry, University Hospital Amsterdam, The Netherlands.
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MeSH Terms
3-Hydroxybutyric Acid
Acyl-CoA Dehydrogenase
Acyl-CoA Dehydrogenases / deficiency*,  urine
Fatty Acids / metabolism*
Hydroxybutyrates / metabolism
Leukocytes / enzymology,  metabolism*
Lipid Metabolism, Inborn Errors / diagnosis,  enzymology*
Mitochondria / metabolism
Octanoic Acids / metabolism
Oxidation-Reduction / drug effects
Palmitates / metabolism
Time Factors
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 Dehydrogenase

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

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