very long-chain
Very-long-chain polyunsaturated fatty acids in the retina: analysis and clinical relevance in physiological and pathological conditions
... being very-long- chain (C24–C36) polyunsaturated fatty acids (four, five or six double bonds, VLC- PUFA) (Aveldano, 1987; Aveldano and Abstract: The retina is one of the vertebrate tissues with the ...
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Very long-chain fatty acids support synaptic structure and function in the mammalian retina
... of very long chain fatty acids-like 4) est une élongase d’acide gras impliquée spécifiquement dans la voie de biosynthèse d’acides gras à très longue chaîne (VLC de longueur de chaîne ≥ 26 atomes de ...
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Interactions of a very long chain fatty acid with model membranes and serum albumin Implications for the pathogenesis of adrenoleukodystrophy
... of very long chain saturated fatty acids (VLCFA), especially the 26-carbon acid, hexacosanoic acid (HA), in membranes and ...acyl chain must then penetrate very deeply into the ...
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Identification of a mutant from Arachis veigae with enhanced seed oleic and very long-chain fatty acid content
... and very long chain (C ≥ 22) fatty acid content was identified from screen- ing 209 accessions of 45 species using gas chromatography ...of very long-chain (C ≥ 22) fatty acids, ...
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Diagnosis of Very Long Chain Acyl-Dehydrogenase Deficiency From an Infant’s Newborn Screening Card
... short chain, medium chain, and very long chain acyl-CoA dehydrogenase deficien- cies, of approximately 1/12 ...cardiomyopathy, very long chain fatty acid ...
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Evaluation of Fourier Transform Infrared Spectroscopy for Diagnosis of Peroxisomal Diseases with Abnormal Very Long Chain Fatty Acid Metabolism
... Peroxisome is one of organelles found in most eukaryotic cells. This organelle plays a role not only in decom- posing hydrogen peroxide, but in β -oxidizing very long chain fatty acids (VLCFAs) ...
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Production of very long chain polyunsaturated omega-3 and omega-6 fatty acids in plants
... of very long chain fatty acids (VLCFAs), including saturated and monounsaturated fatty acids, into glycerolipids, although these phenotypic effects were only observed in a few highly expressing ...
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AdrenoleukodystrophyIncreased plasma content of saturated very long chain fatty acids
... Some heterozygotes showed plasma elevations of very long chain fatty acids t h a t were as marked as those in the hemizygotes, and which we also observed in cultured skin [r] ...
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Recurrent Myoglobinuria as a Presenting Manifestation of Very Long Chain Acyl Coenzyme A Dehydrogenase Deficiency
... of long chain-specific en- zyme, therapy should include avoidance of hypogly- cemic episodes and there may be a rationale for use of a diet low in long chain fatty ...
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Renal response to short- and long-term exercise in very-long-chain acyl-CoA dehydrogenase-deficient (VLCAD−/−) mice
... To test whether the oxidation rate in the kidney of VLCAD −/− mice is influenced by increased energy demand or fasting, we measured the turnover rate of C16-CoA in mice during different conditions. As shown in Figure 1A, ...
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Purification of human very long chain acyl coenzyme A dehydrogenase and characterization of its deficiency in seven patients
... mitochondrial palmitoylcoenzyme A dehydrogenation in liver, heart, skeletal muscle, and skin fibroblasts (89-97, 86-99, 96-99, and 78-87%, respectively). Skin fibroblasts from 26 patients suspected of having a disorder ...
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Essential role of Elovl4 in very long chain fatty acid synthesis, skin permeability barrier function, and neonatal survival
... of long chain fatty acids, suggesting that ELOVL4 may have a similar function (hence the name, ELOVL), and is highly ex- pressed in rod and cone photoreceptor ...of long chain fatty acid) ...
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Membrane microviscosity is increased in the erythrocytes of patients with adrenoleukodystrophy and adrenomyeloneuropathy
... Adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN) are related X-linked disorders characterized by adrenal, gonadal, and nervous system dysfunction. While the pathologic finding common to these tissues appears to ...
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Interaction between peroxisomes and mitochondria in fatty acid metabolism
... The peroxisomal beta oxidation starts also with the con- version of the fatty acid (especially very long chain fatty acids or branched fatty acids) into acyl-CoA. The acyl- CoA may then enter into ...
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Pericarditis as the Presenting Feature of Adrenoleukodystrophy
... of very long chain fatty acids in the serum, brain, and adrenal glands that can lead to neurodevelopmental impairment and decreased adrenal ...of very long chain fatty acids and ...
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Metabolic characterization of the natural progression of chronic hepatitis B
... plasmalogens, very long chain triglyceride spe- cies, and urea cycle intermediates across the progression of the four clinical phases of chronic HBV ...
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Recombinant Cells And Plants For Synthesis Of Very Long Chains Fatty Acid (vlcfa) (Patent WO 2009/156469 A1)
... Very-long-chain fatty acids (VLCFAs) are components of eukaryotic cells and are composed of 20 or more carbons in length (i.e. >C18). VLCFAs are involved in many different physiological functions ...
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Beneficial reward-to-risk action of glucosamine during pathogenesis of osteoarthritis
... of very long chain fatty acids (VLCFA) Recently, our laboratory suggested an interconnec- tion between mitochondria and peroxisome during OA pathogenesis ...
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Increasing medium chain fatty acids production in Yarrowia lipolytica by metabolic engineering
... membrane. Very long chain fatty acids (VLCFA) synthesis occurs in the endoplasmic reticulum membrane and corresponds to the elongation of C16-C18 FA to C20-C26 FA ...acyl chain with ...
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Treatment of cardiomyopathy and rhabdomyolysis in long chain fat oxidation disorders using an anaplerotic odd chain triglyceride
... of long-chain fatty acid oxidation defects (high carbohydrate with medium-even-chain triglycerides and reduced amounts of long-chain fats) fails, in many cases, to pre- vent ...
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