Intermediates in fatty acid oxidation
نویسندگان
چکیده
منابع مشابه
The role of intermediates in mitochondrial fatty acid oxidation.
1. Rat liver mitochondria oxidizing [16-14C]palmitoylcarnitine accumulate saturated long-chain thiester intermediates which may be detected by radio-g.1.c.2. Time-courses of intermediate accumulation display no product-precursor relationships and the end product, measured as [14C]citrate, is produced without a detectable initial lag. 3. A short pulse of [16-14C]palmitoylcarnitine followed by un...
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We describe a method for the diagnosis of mitochondrial fatty acid oxidation disorders that is based on the analysis of acylcarnitine and acyl-coenzyme A (acyl-CoA) esters generated during fatty acid oxidation by permeabilized skin fibroblasts. This method requires only small amounts of cultured fibroblasts with minimal preparation, and no isolation of mitochondrial fractions is necessary. Duri...
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The free fatty acid and total fatty acid profiles in plasma of nine patients with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, two with very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency and two with mild-type multiple acyl-CoA dehydrogenase (MAD-m) deficiency, were analyzed by gas chromatography-mass spectrometry. In the plasma of patients with MCAD deficiency we found increas...
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This over-all equation is thought to consist of the repetition of a series of reactions, each series being initiated by the condensation of malonyl coenzyme A with an unsubstituted straight chain acyl compound to form ,&ketoacyl-S-enzyme (5, 8). A series is then presumably concluded by reactions in which the P-ketoacyl compound is reduced and dehydrated to form an acyl compound 2 carbon atoms l...
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Fatty acid oxidation defects (FAODs) are a group of inborn errors of metabolism that carry a risk for morbidity and mortality. Successful management of these conditions involves early diagnosis, proper management of clinical situations that predisposes to metabolic crisis as well as adequate treatment of a metabolic crisis. The advent of tandem mass spectrometry based newborn screening has redu...
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ژورنال
عنوان ژورنال: Biochemical Journal
سال: 1973
ISSN: 0306-3283
DOI: 10.1042/bj1320061