Long-chain acyl-CoA dehydrogenase
... (EC 1.3.8.8, palmitoyl-CoA dehydrogenase, palmitoyl-coenzyme A dehydrogenase, long-chain acyl ... long-chain-acyl-CoA:(acceptor) 2,3-oxidoreductase, ACADL (gene).) is an enzyme with systematic name long-chain acyl-CoA: ... medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and ... Long-chain+acyl-CoA+dehydrogenase at the U.S. National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology (EC ...
Short-chain acyl-CoA dehydrogenase
... medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and ... Acyl-CoA dehydrogenase Medium-chain acyl-CoA dehydrogenase Butyryl-CoA (also known as butanoyl-CoA) Mahler HR (January 1954). " ... Short-chain acyl-CoA dehydrogenase (EC 1.3.8.1, butyryl-CoA dehydrogenase, butanoyl-CoA dehydrogenase, butyryl dehydrogenase, ... short-chain acyl CoA dehydrogenase, short-chain acyl-coenzyme A dehydrogenase, 3-hydroxyacyl CoA reductase, butanoyl-CoA:( ...
ACADVL
... load and carnitine load on plasma long-chain acylcarnitine levels in mitochondrial very long-chain acyl-CoA dehydrogenase ... This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product ... "acyl-CoA dehydrogenase, very long chain". Strauss AW, Powell CK, Hale DE, Anderson MM, Ahuja A, Brackett JC, Sims HF (Nov 1995 ... Very long-chain specific acyl-CoA dehydrogenase, mitochondrial (VLCAD) is an enzyme that in humans is encoded by the ACADVL ...
Very long-chain acyl-coenzyme A dehydrogenase deficiency
Due to this mutation, effective levels of very long-chain-acyl-CoA-dehydrogenase are low or absent in the body, giving rise to ... A change of the gene that codes for very long-chain-acyl-CoA-dehydrogenase (VLCAD) results in a deficiency or malfunction of ... "Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD)". "VLCAD deficiency , Genetic and Rare Diseases Information Center ( ... Mutations in the ACADVL gene lead to inadequate levels of an enzyme called very long-chain acyl-coenzyme A (CoA) dehydrogenase ...
Mitochondrial trifunctional protein deficiency
"Long-Chain Acyl CoA Dehydrogenase Deficiency: Background, Pathophysiology, Epidemiology". eMedicine. 24 March 2016. Retrieved ... long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), long-chain enoyl-CoA hydratase, and long-chain thiolase activities. ... Avoiding factors that might precipitate condition Glucose Low fat/high carbohydrate nutrition Long-chain acyl-CoA dehydrogenase ... "HADHA hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [Homo ...
Acyl-CoA dehydrogenase
... or long-chain fatty acid acyl-CoA substrates. While different dehydrogenases target fatty acids of varying chain length, all ... "Thermal unfolding of medium-chain acyl-CoA dehydrogenase and iso(3)valeryl-CoA dehydrogenase: study of the effect of genetic ... "Mechanism of activation of acyl-CoA substrates by medium chain acyl-CoA dehydrogenase: interaction of the thioester carbonyl ... Acyl CoA Beta oxidation Thorpe, C.; Kim, J. J. (June 1995). "Structure and Mechanism of Action of the Acyl-CoA Dehydrogenases ...
ACADM
"Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction". The Journal of Biological ... "ACADM - Medium-chain specific acyl-CoA dehydrogenase, mitochondrial precursor - Homo sapiens (Human) - ACADM gene & protein". ... Wang SS, Fernhoff PM, Hannon WH, Khoury MJ (1999). "Medium chain acyl-CoA dehydrogenase deficiency human genome epidemiology ... "Molecular cloning of cDNAs encoding rat and human medium-chain acyl-CoA dehydrogenase and assignment of the gene to human ...
ACADL
... acyl-CoA dehydrogenase, long chain - which is a member of the acyl-CoA dehydrogenase family. The acyl-CoA dehydrogenase family ... "Cardiac hypertrophy in mice with long-chain acyl-CoA dehydrogenase or very long-chain acyl-CoA dehydrogenase deficiency". ... Acyl-CoA dehydrogenase, long chain". Kurtz DM, Tolwani RJ, Wood PA (May 1998). "Structural characterization of the mouse long- ... Acyl-CoA dehydrogenase, long chain is a protein that in humans is encoded by the ACADL gene. ACADL is a gene that encodes LCAD ...
ACAD10
"Identification and characterization of new long chain acyl-CoA dehydrogenases". Molecular Genetics and Metabolism. 102 (4): 418 ... Acyl-CoA dehydrogenase family, member 10 is a protein that in humans is encoded by the ACAD10 gene. This gene encodes a member ... "Entrez Gene: Acyl-CoA dehydrogenase family, member 10". Bian L, Hanson RL, Muller YL, Ma L, Kobes S, Knowler WC, Bogardus C, ... of the acyl-CoA dehydrogenase family of enzymes (ACADs), which participate in the beta-oxidation of fatty acids in mitochondria ...
Very long chain fatty acid
Retrieved 5 January 2013.{{cite web}}: CS1 maint: multiple names: authors list (link) "Very long-chain acyl-CoA dehydrogenase ... A very-long-chain fatty acid (VLCFA) is a fatty acid with 22 or more carbons. Their biosynthesis occurs in the endoplasmic ... doi:10.1016/j.plipres.2006.01.004 "Very-long-chain fatty acids from the animal and plant kingdoms" Rezanka, Tomas Progress in ... Trenkamp, S.; Martin, W.; Tietjen, K. (10 August 2004). "Specific and differential inhibition of very-long-chain fatty acid ...
ACADS
Acyl-CoA dehydrogenase, C-2 to C-3 short chain is an enzyme that in humans is encoded by the ACADS gene. This gene encodes a ... The coding sequence of this gene is 1239 bp long. The encoded protein has 412 amino acids, and its size is 44.3 kDa (Human) or ... "Entrez Gene: Acyl-CoA dehydrogenase, C-2 to C-3 short chain". Tein I, Elpeleg O, Ben-Zeev B, Korman SH, Lossos A, Lev D, Lerman ... As short-chain acyl-CoA dehydrogenase is involved in beta-oxidation, a deficiency in this enzyme is marked by an increased ...
ACAD9
"Acyl-CoA dehydrogenase 9 (ACAD 9) is the long-chain acyl-CoA dehydrogenase in human embryonic and fetal brain". Biochemical and ... typically C16-acylCoA and longer. It has been observed that ACAD9 can catalyze acyl-CoAs with very long chains. The specific ... "Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients". ... Acyl-CoA dehydrogenase family member 9, mitochondrial is an enzyme that in humans is encoded by the ACAD9 gene. Mitochondrial ...
Jamaican vomiting sickness
... it interferes with the transport of long-chain fatty acids into the mitochondria. Also, it inhibits acyl-CoA dehydrogenases, so ...
Mitochondrial trifunctional protein
CoA) hydratase, long-chain 3-hydroxy acyl-coenzyme A dehydrogenase and long-chain 3-ketoacyl CoA thiolase. Fatty acid beta- ... "Long-Chain Acyl CoA Dehydrogenase Deficiency: eMedicine Pediatrics: Genetics and Metabolic Disease". Retrieved 2009-07-11. Wang ...
List of diseases (A)
... deficiency of Acyl-CoA dehydrogenase, short chain, deficiency of Acyl-CoA dehydrogenase, very long chain, deficiency of Acyl- ... promyelocytic leukemia Acute renal failure Acute respiratory distress syndrome Acute tubular necrosis Acyl-CoA dehydrogenase, ... CoA oxidase deficiency Adactylia unilateral dominant ADAM complex Adams-Nance syndrome Adams-Oliver syndrome Addison's disease ... vitiligo Alpers disease Alpha 1-antitrypsin deficiency Alpha-2 deficient collagen disease Alpha-ketoglutarate dehydrogenase ...
List of causes of hypoglycemia
Trimethoprim Triple A syndrome Tumors Tyrosinaemia type 1 Urea cycle disorder Uremia Very-long-chain acyl-CoA dehydrogenase ... Reye syndrome Ritonavir Saquinavir Sepsis Septic shock Severe hepatitis Sheehan syndrome Short-chain acyl-CoA dehydrogenase ... deficiency Maple syrup urine disease Mcquarrie type infantile idiopathic hypoglycemia Medium chain acyl-CoA dehydrogenase ... Disorders of fatty acid oxidation Medium chain acylCoA dehydrogenase deficiency (MCAD) Familial Leucine sensitive hypoglycemia ...
ETFA
... displays decreased thermal stability and is overrepresented in very-long-chain acyl-CoA dehydrogenase-deficient patients with ... A crystal structure of the complex of one of its interactors, medium-chain acyl-CoA dehydrogenase (MCAD; gene name ACADM) has ... "Acyl-CoA dehydrogenases, electron transfer flavoprotein and electron transfer flavoprotein dehydrogenase". Biochemical Society ... Crane FL, Beinert H (September 1954). "A Link Between Fatty Acyl CoA Dehydrogenase and Cytochrome C: A New Flavin Enzyme". ...
ETFB
... displays decreased thermal stability and is overrepresented in very-long-chain acyl-CoA dehydrogenase-deficient patients with ... A crystal structure of the complex of one of its interactors, medium-chain acyl-CoA dehydrogenase (MCAD; gene name ACADM) has ... "Acyl-CoA dehydrogenases, electron transfer flavoprotein and electron transfer flavoprotein dehydrogenase". Biochemical Society ... Crane FL, Beinert H (September 1954). "A Link Between Fatty Acyl CoA Dehydrogenase and Cytochrome C: A New Flavin Enzyme". ...
Fatty acid
ISBN 978-1-118-16945-2. Roth, Karl S. (2013-12-19). "Medium-Chain Acyl-CoA Dehydrogenase Deficiency". Medscape. Beermann, C.; ... Long-chain fatty acids (LCFAs) are fatty acids with aliphatic tails of 13 to 21 carbons. Very long chain fatty acids (VLCFAs) ... The cytosolic acetyl-CoA is carboxylated by acetyl CoA carboxylase into malonyl-CoA, the first committed step in the synthesis ... Malonyl-CoA is then involved in a repeating series of reactions that lengthens the growing fatty acid chain by two carbons at a ...
Carnitine
... the fatty acyl-CoA dehydrogenases for short, medium, long, and very long acyl chains, and related enzymes. PPARα functions as a ... the fatty acyl group is transferred from fatty acyl-carnitine to coenzyme A, regenerating fatty acyl-CoA and a free carnitine ... displacing AMP to form thioester fatty acyl-CoA. In the second reaction, acyl-CoA is transiently attached to the hydroxyl group ... The first reaction of the carnitine shuttle is a two-step process catalyzed by a family of isozymes of acyl-CoA synthetase that ...
Acyl-CoA
These enzymes are better equipped to oxidize Acyl-CoA with long chains that the mitochondria cannot handle. Beta oxidation ... Beta oxidation of acyl-CoA occurs in four steps. 1. Acyl-CoA dehydrogenase catalyzes dehydrogenation of the acyl-CoA, creating ... The latter conversion is mediated by acyl-CoA synthase" acyl-P + HS-CoA → acyl-S-CoA + Pi + H+ Three types of acyl-CoA ... For example, the substrates for medium chain acyl-CoA synthase are 4-11 carbon fatty acids. The enzyme acyl-CoA thioesterase ...
Acyl-CoA thioesterase 9
... as opposed to long-chain acyl-CoA synthetases, which ligate fatty acids to CoA, to produce the CoA ester. The role of the ACOT ... In the mitochondria, acyl-CoA esters are involved in the acylation of mitochondrial NAD+ dependent dehydrogenases; because ... Acyl-CoA thioesterase 9 is a protein that is encoded by the human ACOT9 gene. It is a member of the acyl-CoA thioesterase ... These enzymes have also been referred to in the literature as acyl-CoA hydrolases, acyl-CoA thioester hydrolases, and palmitoyl ...
Fatty acid desaturase
Acyl-CoA dehydrogenases are enzymes that catalyze formation of a double bond between C2 (α) and C3 (β) of the acyl-CoA ... Abedi E, Sahari MA (September 2014). "Long-chain polyunsaturated fatty acid sources and evaluation of their nutritional and ... Thorpe C, Kim JJ (June 1995). "Structure and mechanism of action of the acyl-CoA dehydrogenases". FASEB Journal. 9 (9): 718-25 ... Plant stearoyl-acyl-carrier-protein desaturase (EC 1.14.19.1), an enzyme that catalyzes the introduction of a double bond at ...
List of disorders included in newborn screening programs
Short-chain hydroxy Acyl-CoA dehydrogenase deficiency (SCHAD) Long-chain acyl-CoA dehydrogenase deficiency (LCAD) Multiple acyl ... 1 in 75,000 Medium-chain acyl-CoA dehydrogenase deficiency (MCAD) > 1 in 25,000 Very-long-chain acyl-CoA dehydrogenase ... Medium/short-chain L-3-hydroxy acyl-CoA dehydrogenase deficiency Medium-chain ketoacyl-CoA thiolase deficiency Dienoyl-CoA ... 1 in 100,000 Inborn errors of fatty acid metabolism Long-chain hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) > ...
Type 2 diabetes
April 2011). "Toxic response caused by a misfolding variant of the mitochondrial protein short-chain acyl-CoA dehydrogenase". ... Long-term complications from high blood sugar include heart disease, strokes, diabetic retinopathy which can result in ... Haw JS, Galaviz KI, Straus AN, Kowalski AJ, Magee MJ, Weber MB, Wei J, Narayan KM, Ali MK (December 2017). "Long-term ... A 2017 review found that, long term, lifestyle changes decreased the risk by 28%, while medication does not reduce risk after ...
Fatty-acid metabolism disorder
Dehydrogenase Deficiency (GAII & MADD) 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency (HMG deficiency) Very long-chain acyl- ... Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (LCHAD deficiency) Medium-chain acyl-coenzyme A dehydrogenase ... Short-chain acyl-coenzyme A dehydrogenase deficiency (SCAD deficiency) 3-hydroxyacyl-coenzyme A dehydrogenase deficiency (M/ ... 4 Dienoyl-CoA Reductase Deficiency Electron Transfer Flavoprotein (ETF) ...
Tambjamine
... introduces a double bond into a fatty acyl side chain. TamH then carries out the reduction of the CoA-ester to form an aldehyde ... AfaA is hypothesized to activate long-chain fatty acids while the predicted dehydrogenase, TamT, ... The biosynthesis of tambjamine YP1 first involves the incorporation of proline, malonyl Co-A, and serine to form 4-methoxy-2,2 ... the majority have short alkyl chains connected to the enamine nitrogen. This group of alkaloids have been isolated from marine ...
Ethanol metabolism
The first is acyl-CoA synthetase short-chain family member 2 ACSS2 (EC 6.2.1.1). The second enzyme is acetyl-CoA synthase 2 ( ... "ALDH2 aldehyde dehydrogenase 2 family member [Homo sapiens (human)] - Gene - NCBI". www.ncbi.nlm.nih.gov. Retrieved 2018-04-28 ... "Hominids adapted to metabolize ethanol long before human-directed fermentation". Proceedings of the National Academy of ... "ACSS2 acyl-CoA synthetase short chain family member 2 [Homo sapiens (human)] - Gene - NCBI". www.ncbi.nlm.nih.gov. Retrieved ...
List of OMIM disorder codes
SCARB2 Acyl-CoA dehydrogenase, long chain, deficiency of; 201460; ACADL Acyl-CoA dehydrogenase, medium chain, deficiency of; ... ACADM Acyl-CoA dehydrogenase, short chain, deficiency of; 201470; ACADS Adenocarcinoma of lung, response to tyrosine kinase ... AKAP9 Long QT syndrome-3; 603830; SCN5A Long QT syndrome-4; 600919; ANK2 Long QT syndrome-7; 170390; KCNJ2 Long QT syndrome-9; ... TGFBR2 Long QT syndrome 12; 612955; SNT1 Long QT syndrome 13; 613485; KCNJ5 Long QT syndrome-1; 192500; KCNQ1 Long QT syndrome- ...
Astrocyte
"Misfolding of short-chain acyl-CoA dehydrogenase leads to mitochondrial fission and oxidative stress". Molecular Genetics and ... Pain processing is no longer seen as a repetitive relay of signals from body to brain, but as a complex system that can be up- ... Long-term potentiation: Scientists debate whether astrocytes integrate learning and memory in the hippocampus. Recently it has ... In their results they were able to see that astrocytes had a direct role in Long-term potentiation with the mixed culture ( ...
Very-long-chain acyl-CoA dehydrogenase
... (EC 1.3.8.9, ACADVL (gene).) is an enzyme with systematic name very-long-chain acyl-CoA: ... "Structural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenase ... Very-long-chain+acyl-CoA+dehydrogenase at the U.S. National Library of Medicine Medical Subject Headings (MeSH) Portal: Biology ... I. Purification and properties of very-long-chain acyl-coenzyme A dehydrogenase". The Journal of Biological Chemistry. 267 (2 ...
Medium-chain acyl-CoA dehydrogenase
... medium-chain, and long-chain acyl-CoA dehydrogenases from rat liver mitochondria. Isolation of the holo- and apoenzymes and ... acyl dehydrogenase (ambiguous), fatty-acyl-CoA dehydrogenase (ambiguous), acyl CoA dehydrogenase (ambiguous), general acyl CoA ... Medium-chain acyl-CoA dehydrogenase (EC 1.3.8.7, fatty acyl coenzyme A dehydrogenase (ambiguous), acyl coenzyme A dehydrogenase ... dehydrogenase (ambiguous), medium-chain acyl-coenzyme A dehydrogenase, acyl-CoA:(acceptor) 2,3-oxidoreductase (ambiguous), ...
Acyl-CoA dehydrogenase (NADP+)
I The mechanism of elongation of long-chain fatty acids by acetyl-CoA". Biochim. Biophys. Acta. 164 (3): 498-517. doi:10.1016/ ... In enzymology, an acyl-CoA dehydrogenase (NADP+) (EC 1.3.1.8) is an enzyme that catalyzes the chemical reaction acyl-CoA + ... crotonyl-CoA reductase, and acyl-CoA dehydrogenase (NADP+). As of late 2007, only one structure has been solved for this class ... Other names in common use include 2-enoyl-CoA reductase, dehydrogenase, acyl coenzyme A (nicotinamide adenine dinucleotide, ...
Erucic acid
... is broken down into shorter-chain fatty acids in the human liver by the long-chain acyl CoA dehydrogenase enzyme. ... although the long-term use of Lorenzo's oil (oleic acid and erucic acid) in the treatment of adrenoleukodystrophy or ... While there are reports of toxicity from long-term use of Lorenzo's oil (which contains erucic acid and other ingredients), ... Erucic acid is produced by elongation of oleic acid via oleoyl-coenzyme A and malonyl-CoA. ...
Beta oxidation
If the fatty acyl-CoA has a long chain, then the carnitine shuttle must be utilized: Acyl-CoA is transferred to the hydroxyl ... is not an appropriate substrate for acyl CoA dehydrogenase, or enoyl CoA hydratase: If the acyl CoA contains a cis-Δ3 bond, ... Cn-acyl-CoA + FAD + NAD+ + H 2O + CoA → Cn-2-acyl-CoA + FADH 2 + NADH + H+ + acetyl-CoA Free fatty acids cannot penetrate any ... The final cycle produces two separate acetyl CoAs, instead of one acyl CoA and one acetyl CoA. For every cycle, the Acyl CoA ...