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Intermediates of unsaturated fatty acid oxidation are incorporated in triglycerides but not in phospholipids in tissues from patients with mitochondrial β‐oxidation defects

The fatty acid composition was determined of liver, skeletal muscle and heart obtained post mortem from patients with medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD), multiple acyl‐CoA dehydrogenase deficiency (MADD) and very long‐chain acyl‐CoA dehydrogenase deficiency (VLCADD). Increased am...

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Published in:Journal of inherited metabolic disease 2001-06, Vol.24 (3), p.337-344
Main Authors: Onkenhout, W., Venizelos, V., Scholte, H. R., Klerk, J. B. C., Poorthuis, B. J. H. M.
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container_title Journal of inherited metabolic disease
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creator Onkenhout, W.
Venizelos, V.
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Poorthuis, B. J. H. M.
description The fatty acid composition was determined of liver, skeletal muscle and heart obtained post mortem from patients with medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD), multiple acyl‐CoA dehydrogenase deficiency (MADD) and very long‐chain acyl‐CoA dehydrogenase deficiency (VLCADD). Increased amounts of 4‐decenoic acid 10:1(n−6), 5‐dodecenoic acid 12:1(n−7), 5‐tetradecenoic acid 14:1(n−9), 5,8‐tetradecadienoic acid 14:2(n−6) and 7,10‐hexadecadienoic acid 16:2(n−6)—intermediates of unsaturated fatty acid oxidation—were found. Fractionation into different lipid classes showed that these fatty acids were exclusively present in the triglyceride fraction. They could not be detected in the free fatty acid fraction or in the phospholipid fraction. Our results suggest that intermediates of unsaturated fatty acid oxidation that accumulate as a consequence of MCADD, MADD and VLCADD are transported to the endoplasmic reticulum for esterification into neutral glycerolipids. The pattern of accumulation is characteristic for each disease, which makes fatty acid analysis of total lipid of post‐mortem tissues a useful tool in the detection of mitochondrial fatty acid oxidation defects in patients who died unexpected, for example with sudden infant death syndrome.
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ispartof Journal of inherited metabolic disease, 2001-06, Vol.24 (3), p.337-344
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source Springer Journals; Wiley-Blackwell Read & Publish Collection
subjects Acyl-CoA Dehydrogenase, Long-Chain - deficiency
Biological and medical sciences
Endoplasmic Reticulum - metabolism
Errors of metabolism
Esterification
Fatal Outcome
Fatty Acids, Unsaturated - analysis
Fatty Acids, Unsaturated - metabolism
Female
Humans
Infant
Infant, Newborn
Lipids (lysosomal enzyme disorders, storage diseases)
Liver - chemistry
Liver - metabolism
Male
Medical sciences
Metabolic diseases
Mitochondrial Diseases - metabolism
Muscle, Skeletal - chemistry
Muscle, Skeletal - metabolism
Myocardium - chemistry
Myocardium - metabolism
Oxidation-Reduction
Phospholipids - metabolism
Triglycerides - metabolism
title Intermediates of unsaturated fatty acid oxidation are incorporated in triglycerides but not in phospholipids in tissues from patients with mitochondrial β‐oxidation defects
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