| Quantitative measurement of total and free 3-hydroxy fatty acids in serum or plasma samples: short-chain 3-hydroxy fatty acids are not esterified. | |
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MedLine Citation:
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PMID: 11117435 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Diagnostic protocols for disorders of mitochondrial fatty acid oxidation (FAO) generally include the measurement of plasma acylcarnitines. Many biochemical intermediates of FAO resulting from a metabolic block require carnitine conjugation for transport out of the mitochondria, and so occur as fatty acid carnitine conjugates in the blood. Both short- and long-chain acylcarnitines are generally determined, and this procedure has a critical role to play in the diagnosis of disorders of the very long-chain, medium-chain and short-chain acyl-CoA dehydrogenase defects. Less is known about the utility of acylcarnitines for the measurement of the various chain length intermediates of the 3-hydroxyacyl-CoA dehydrogenase steps of beta-oxidation. This study utilizes stable-isotope dilution gas chromatography-mass spectrometry to determine the serum or plasma concentrations of free 3-hydroxy fatty acids (3-OHFAs) of chain lengths C6 to C16. The 3-OHFA concentrations are determined in samples from normal individuals, hyperketotic individuals and patients with long-chain L-3-hydroxyacyl-CoA dehydrogenase and short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiencies, both before and after hydrolysis. The results of the study indicate the relative amounts of conjugated intermediates of all chain lengths. Long-chain 3-OHFAs (C14 and C16) are found in elevated concentrations after hydrolysis, whereas short-chain and medium-chain 3-OHFAs (C6 to C12) show no difference in concentrations between the two samples in all subjects tested, suggesting that only long-chain 3-hydroxy species form conjugates. This finding has important implications for the use of the acylcarnitine assay for the diagnosis of defects involving short-chain and medium-chain 3-hydroxy fatty acids. |
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Authors:
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P M Jones; A B Burlina; M J Bennett |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of inherited metabolic disease Volume: 23 ISSN: 0141-8955 ISO Abbreviation: J. Inherit. Metab. Dis. Publication Date: 2000 Nov |
Date Detail:
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Created Date: 2000-12-14 Completed Date: 2001-03-08 Revised Date: 2007-03-21 |
Medline Journal Info:
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Nlm Unique ID: 7910918 Medline TA: J Inherit Metab Dis Country: Netherlands |
Other Details:
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Languages: eng Pagination: 745-50 Citation Subset: IM |
Affiliation:
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Department of Pathology, University of Texas, Southwestern Medical Center, Dallas, USA. pjones@childmed.dallas.tx.us |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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3-Hydroxyacyl CoA Dehydrogenases
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blood,
deficiency* Acyl-CoA Dehydrogenase Acyl-CoA Dehydrogenase, Long-Chain / blood, deficiency* Esterification Fatty Acids, Nonesterified / blood* Humans |
| Chemical | |
Reg. No./Substance:
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0/Fatty Acids, Nonesterified; EC 1.1.1.211/long-chain 3-hydroxyacyl CoA dehydrogenase; EC 1.1.1.35/3-Hydroxyacyl CoA Dehydrogenases; EC 1.3.99.13/Acyl-CoA Dehydrogenase, Long-Chain; 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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