fatty acyl coenzyme A
The multiple acyl coenzyme A dehydrogenation disorders, glutaric aciduria type II and ethylmalonic adipic aciduria Mitochondrial fatty acid oxidation, acyl coenzyme A dehydrogenase, and electron transfer flavoprotein activities in fibroblasts
... multiple acyl-coenzyme A (CoA) dehydrogenation disorders (MAD) include severe (S) and mild (M) variants, glutaric aciduria type II (MAD:S) and ethylmalonic-adipic aciduria ...mitochondria, acyl-CoA ...
10
Relationship between unusual hepatic acyl coenzyme A profiles and the pathogenesis of Reye syndrome
... reticulum. The results suggest that pathogenesis in Reye syndrome stems from generalized mitochondrial damage resulting in accumulation of acyl CoA esters. High levels of these compounds lead to inhibition of ...
8
Prolonged QTc Interval in Association With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency
... of fatty acid oxida- tion, with an incidence of 1 in 15 000 to 1 in 20 000 ...mitochondrial fatty acid b -oxidation of medium- chain ...encodes acyl-coenzyme A dehydrogenase for medium-chain ...
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Complementation analysis of fatty acid oxidation disorders
... with fatty acid oxidation ...chain acyl-coenzyme A (CoA) dehydrogenase deficiency (115%), medium chain acyl-CoA dehydrogenase deficiency (18%), long-chain acyl-CoA dehydrogenase ...
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Changes in peroxisome proliferator-activated receptor alpha target gene expression in peripheral blood mononuclear cells associated with non-alcoholic fatty liver disease
... ACADVL: Acyl-coenzyme A dehydrogenase 2 long chain; ALP: Alkaline phosphatase; ALT: Alanine aminotransferase; AST: Aspartate aminotransferase; BMI: Body mass index; CN: Control group; CPT1A: Carnitine ...
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Short-Chain Fatty Acid Activation by Acyl-Coenzyme A Synthetases Requires SIR2 Protein Function in Salmonella enterica and Saccharomyces cerevisiae
... short-chain fatty acid metabolism obtained after treatment with CobB/NAD ⫹ was equiva- beyond activation is unlikely since the lack of sirtuin lent to the level of enzyme activity measured in cell- function was ...
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Essential amino acid ratios and mTOR affect lipogenic gene networks and miRNA expression in bovine mammary epithelial cells
... ACSL1, Acyl-CoA synthetase long-chain family member 1; ACSS2, Acyl-CoA synthetase short-chain family member 2; AGPAT6, 1-acylglycerol-3-phosphate O-acyltransferase 6; DGAT1, diacylglycerol acyltransferase ...
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Purification of human very long chain acyl coenzyme A dehydrogenase and characterization of its deficiency in seven patients
... measuring acyl- coenzyme A dehydrogenation activities, overall palmitic acid oxidation, and VLCAD protein synthesis using pulse-chase, further confirming the diagnosis of VLCAD ...long-chain fatty ...
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Physiological Role of Acyl Coenzyme A Synthetase Homologs in Lipid Metabolism in Neurospora crassa
... six fatty ACSs; two (FaaA and FaaB) are homologs to the Faa proteins of ...six fatty ACSs were interro- gated for their involvement in FA activation for degradation by assessing induction of the associated ...
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PPARα protein expression was increased by four weeks of intermittent hypoxic training via AMPKα2-dependent manner in mouse skeletal muscle
... of fatty acid ...chain acyl coenzyme A dehydrogenase (MCAD) mRNA, the key enzyme for fatty acid oxidation and one of the PPAR α target genes, was also measured in skeletal mus- cles after the ...
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Modulation Peroxisome Proliferators Activated Receptor alpha (PPAR α) and Acyl Coenzyme A: Cholesterol Acyltransferase1 (ACAT1) Gene expression by Fatty Acids in Foam cell
... bind fatty acids, only PPAR is completely accepted as a fatty acid-regulated nuclear ...in fatty acids catabolism [9], ketone bodies synthesis [10], insulin effects [11] and lipoprotein assem- bly ...
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Recurrent Myoglobinuria as a Presenting Manifestation of Very Long Chain Acyl Coenzyme A Dehydrogenase Deficiency
... (SG Kahler, Duke University Children’s Hospital, Durham, NC). She was started on a low-fat diet supplemented with carnitine, 3 g/d. The CPK level decreased to normal after 3 weeks. One month later, she experienced muscle ...
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Child Neurology: Medium-chain acyl-coenzyme A dehydrogenase deficiency
... second coenzyme A molecule, which attacks at the b-carbon to produce acetyl-CoA and a fatty acyl-CoA that is 2 carbons shorter and will undergo further oxidation until reduced entirely to ...
5
The endogenous molecular clock orchestrates the temporal separation of substrate metabolism in skeletal muscle
... Acadm: acyl-Coenzyme A dehydrogenase, medium chain; Acly: ATP citrate lyase; Acot7: acyl-CoA thioesterase 7; Acot9: acyl-CoA thioesterase 9; Acsl1: acyl-CoA synthetase long-chain family ...
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Familial Reye-Like Syndrome: A Presentation of Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency
... Medium-chain acyl-coenzyme A (CoA) dehydro- genase deficiency is a recently described, autosomal recessive disorder of fatty acid oxidation that has a superficial similarity to Reye synd[r] ...
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Neonatal Screening for Very Long-Chain Acyl-CoA Dehydrogenase Deficiency: Enzymatic and Molecular Evaluation of Neonates With Elevated C14:1-Carnitine Levels
... for fatty acid oxidation defects, including very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD), have been implemented recently in a num- ber of European countries, as well as in ...
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Metabolic engineering of microbes for branched-chain biodiesel production with low-temperature property
... to fatty acid methyl esters (FAMEs) or ...presented fatty acid short-chain ester (FASE) biosynthesis as an improvement of the low- temperature properties of traditional ...of fatty acyl-CoAs ...
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Fatty alcohol production in Lipomyces starkeyi and Yarrowia lipolytica
... I fatty acid synthase in conjunction with tesA, fadD and the far gene (Maqu_2220 from ...the fatty alcohol production in ...I fatty acid synthesis system commonly found in mammals and fungi can be ...
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Grevengoed_unc_0153D_15339.pdf
... heart. Fatty acid oxidation becomes the dominant source of energy, and membranes change to increase more linoleate and ...use fatty acids for energy may be important as the newborn transitions through the ...
137
Gradients in compositions in the starchy endosperm of wheat have implications for milling and processing
... free fatty acids (Gonzáles-Thuillier et ...the acyl groups esterified to polar lipids and ...total fatty acids, including free fatty acids and fatty acids from acyl lipids) shows ...
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