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Acyl Coenzyme ADiacylglycerol O-AcyltransferaseSterol O-AcyltransferaseAcyltransferasesCoenzyme ASubstrate SpecificityCoenzyme A LigasesAcyl-CoA OxidaseDiazepam Binding InhibitorKineticsFatty AcidsPalmitoyl Coenzyme ATriazenesMolecular Sequence DataCholesterol EstersAmino Acid SequenceEsterificationMicrosomesAcyl-CoA DehydrogenasesAcyl-CoA DehydrogenaseLiverPalmitic AcidsOleic AcidsTriglyceridesLipid MetabolismCholesterolAcylationCatalysisBinding SitesOxidoreductasesOxidation-ReductionCarnitine O-PalmitoyltransferaseMicrosomes, LiverModels, MolecularMicrobodiesLipidsEscherichia coliFatty Acids, UnsaturatedAcetyl Coenzyme AHydrolysisDietary FatsProtein BindingHydrogen-Ion ConcentrationCatalytic DomainCoenzyme A-TransferasesRecombinant ProteinsCrystallography, X-RayStructure-Activity RelationshipMolecular StructurePantetheineSequence Homology, Amino AcidHydroxymethylglutaryl CoA ReductasesProtein ConformationBase SequenceProtein Structure, TertiaryEstersBacterial ProteinsRNA, MessengerAdipose TissueMutationCloning, MolecularMutagenesis, Site-DirectedPantothenic AcidModels, ChemicalPhosphorylationPhosphatidylcholinesChromatography, High Pressure LiquidAdenosine TriphosphateStereoisomerismPhospholipidsTemperatureMagnetic Resonance SpectroscopyMice, Inbred C57BLMolecular WeightSequence AlignmentElectrophoresis, Polyacrylamide GelMolecular ConformationEnzyme ActivationCattleMass SpectrometryCell LineAcetatesChromatography, Thin LayerMalonyl Coenzyme AEscherichia coli ProteinsIsoenzymesPeptidesSaccharomyces cerevisiaeAmino AcidsModels, BiologicalNADEnzyme InhibitorsCarnitineEnzyme StabilityProtein Structure, SecondaryAlcohol OxidoreductasesBiological TransportCarrier ProteinsCell MembraneOligopeptides
SynthetaseEnzymeDehydrogenaseOxidationCoenzymeSynthasePathwaysDiacylglycerolEnzymesBiosynthesisMitochondrial energyMitochondriaSpecificityThioesterVitroDehydrogenationLipid synthesisSynthesisMoietyKetone bodiesElectronResiduesReactionArachidonateProteinMCADPhosphorylationAmino acidCholesterolAccumulationDefectsMoleculePathophysiologyInsulinEfficiencyUpstreamReactionsMetabolismCatalyzeCatabolismPreferenceExcessOxygenActivityProductFreeBackboneTransferMechanisms
Synthetase8
  • In the cytosol, a saturated, straight-chain fatty acid molecule with no double bonds is activated by the action of fatty acyl-CoA synthetase to form its corresponding acyl-CoA. (medscape.com)
  • Acyl-CoA synthetase family member 3, mitochondrial (EC 6.2.1. (joplink.net)
  • Results showed that the three fibrates exhibited very different effects on the hepatic levels of 2-PPA- S -acyl CoA (2-PPA-CoA) in vivo, even though all three significantly increased acyl-CoA synthetase activity in vitro in liver homogenate. (aspetjournals.org)
  • Acyl-CoA synthetase-4, a new regulator of mTOR and a potential therapeutic target for enhanced estrogen receptor function in receptor-positive and -negative. (oncotarget.com)
  • Although the role of acyl-CoA synthetase 4 (ACSL4) in mediating an aggressive phenotype is well accepted, there is little evidence as to the early steps through which ACSL4 increases tumor growth and progression. (oncotarget.com)
  • Recently, an Acyl Activating Enzyme 3 (AAE3), encoding an oxalyl-CoA synthetase, was identified in Arabidopsis. (usda.gov)
  • Mtb FadD2 is a fatty acyl-coenzyme A (CoA) synthetase that modifies the cell envelope and plays an important role in reducing Mtb susceptibility to pyrazinoic acid (POA). (bvsalud.org)
  • Bempedoic acid and its active metabolite, ESP15228, require coenzyme A (CoA) activation by very long?chain acyl-CoA synthetase 1 (ACSVL1) to ETC-1002-CoA and ESP15228-CoA, respectively. (medscape.com)
Enzyme15
  • Entry into the beta-oxidation cycle requires the action of acyl-CoA dehydrogenase, the first enzyme in the sequence, which removes electrons from the alpha-carbon and the beta-carbon, introducing a double bond. (medscape.com)
  • Risk stratification by residual enzyme activity after newborn screening for medium-chain acyl-CoA dehyrogenase deficiency: data from a cohort study. (medscape.com)
  • The deprotonated 3S-C8-CoA was clearly located within the active-site cleft of the enzyme. (rcsb.org)
  • The ketone βOHB enters the mitochondrion where the enzyme BDH1 converts it into acetoacetate, whose products serve as substrates for the citric acid cycle. (the-scientist.com)
  • In this study we report the discovery of oxalyl-CoA decarboxylase which is an enzyme that catalyzes the second step in a previously uncharacterized pathway of oxalate degradation. (usda.gov)
  • Biochemical analysis showed that this enzyme converts oxalyl-CoA into formyl-CoA and CO2. (usda.gov)
  • This enzyme breaks down oxalyl-CoA, the product of AtAAE3, into formyl-CoA and CO2. (usda.gov)
  • This reduction in AtOXC activity resulted in an increase in the accumulation of oxalate and the enzyme substrate, oxalyl-CoA. (usda.gov)
  • The enzyme from the moth Spodoptera littoralis prefers 13:0 and 14:0 CC substrates. (expasy.org)
  • Hydroxymethylglutaryl-CoA synthase 1 and 2 (HMG-CoA synthase 1 and 2) The first enzyme in the lanosterol pathway catalyses the synthesis of the metabolite (S)-3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) from condensation and acyl group transfer of substrates acetyl CoA and acetoacetyl CoA. (davidgessner.com)
  • To produce energy G-6-PO 4 (derived from monosaccharides from dietary CHO or produced from glycogen degradation by glycogenolysis) is converted by a series of enzyme reactions in the glycolytic pathway to form pyruvate or lactic acid, then to acetyl-CoA, which is also produced from fatty acid oxidation and degradation of the carbon skeleton of glucogenic amino acids ( Table 17.1 ). (oncohemakey.com)
  • Moreover, the FAO enzyme very-long-chain acyl-CoA dehydrogenase physically interacted with TFP, thereby creating a multifunctional energy protein complex. (oroboros.at)
  • 2 We also have to keep in mind that 5-hydroxydecanonate is not a selective inhibitor of mitochondrial adenosine triphosphate sensitive K + channels anymore because it is a substrate for the enzyme acyl-CoA synthethase in the electron transport chain of mitochondria, 4 and it is capable of playing a role as an inhibitor of sarcolemmal adenosine triphosphate sensitive K + channels. (asahq.org)
  • ACL is an enzyme upstream of 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) reductase in the cholesterol biosynthesis pathway. (medscape.com)
  • This enzyme group includes all the enzymes that introduce double bonds into substrates by direct dehydrogenation of carbon-carbon single bonds. (bvsalud.org)
Dehydrogenase15
Oxidation7
  • Oxidation of the hydroxyl substituent group on the beta-carbon creates an inherently unstable beta-ketoacyl-CoA compound. (medscape.com)
  • Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders. (medscape.com)
  • To produce ATP, fatty acids are first converted into acylcarnitines, which are converted back to fatty-acyl-CoA and enter the β-oxidation cycle inside mitochondria. (the-scientist.com)
  • b) They transfer hydrogen from one substrate to another by oxidation-reduction reac-tion not involving a respiratory chain (shown in Fig. 12.4. (biologydiscussion.com)
  • Mitochondria supply energy for contractile function via the oxidation of fuel substrates, and complete control of this system is indispensable to maintain cardiac efficiency. (wvu.edu)
  • Fatty acids enter the mitochondria via the carnitine transport cycle (medium chain fatty acids enter independently of carnitine) into the β-oxidation spiral in which fatty acids, via a series of enzymes, produce acetyl-CoA and electron carriers. (oncohemakey.com)
  • VLCAD is a homodimer containing a FAD cofactor that is the first step in the mitochondrial matrix for oxidation of the acyl-CoA substrate. (oroboros.at)
Coenzyme2
  • PHA synthase PhaC forms an active dimer to polymerize acyl moieties from the substrate acyl-coenzyme A (CoA) into PHA polymers. (afpm.org.my)
  • In 1961, Childs et al published the earliest clinical report of a patient who was ultimately found to be affected by a deficiency of propionyl coenzyme A (CoA) carboxylase (ie, propionic acidemia). (medscape.com)
Synthase2
  • The minimal composition of a type I PKS module consists of an acyltransferase (AT) domain, which is responsible for choosing the building block to be used, a keto synthase (KS) domain, which catalyzes the C-C bond formation and an acyl carrier protein (ACP) domain, also known as thiolation domain. (wikipedia.org)
  • FAE ( Fatty Acid Elongation ) like genes take part in the biosynthesis of VLCFAs, growth regulation, and stress responses, and are further comprised of KCS ( Ketoacyl-CoA synthase ) and ELO ( Elongation Defective Elongase ) sub-gene families. (nature.com)
Pathways4
  • Before entering lipid synthesis pathways, different kinds of acyl-CoA transporters which acts as the major carrier of acyl-CoAs, such as fatty acid binding protein (FABP), sterol carrier protein 2 (SCP2) and acyl-CoA-binding protein (ACBP), would activate and convert fatty acids to fatty acyl-CoA esters via a reaction catalyzed by fatty acyl-CoA synthetase and transported to various metabolic locations [ 11 ]. (biomedcentral.com)
  • Generally, TAG biosynthesis takes place in the endoplasmic reticulum, and TAG assembly can be divided into acyl-CoA-dependent and acyl-CoA independent pathways [10]. (researchsquare.com)
  • A series of studies were conducted to explore the inductive potential of different fibric acid derivatives on the two alternative metabolic activation pathways of 2-phenylpropionic acid (2-PPA) (a model substrate for profen drugs), namely acyl-CoA formation and acyl glucuronidation, in vivo in rats, and to evaluate whether such treatment could potentially modulate the covalent binding of profens to hepatic protein. (aspetjournals.org)
  • 18 - 21 However, MCTs rapidly cross the mitochondrial membrane and enter metabolic pathways to be beta-oxidized, producing a larger amount of acetyl-CoA than carbohydrates (CHOs). (jomes.org)
Diacylglycerol3
  • DG(i-18:0/21:0/0:0) is also a substrate of diacylglycerol kinase. (hmdb.ca)
  • Diacylglycerol acyltransferases (DGATs) catalyze the final acylation of sn-1, 2-diacylglycerol (DAG) to form TAG, which is the last and limiting step in the acyl-CoA dependent TAG formation pathway [11]. (researchsquare.com)
  • Catalyzes the terminal and only committed step in triacylglycerol synthesis by using diacylglycerol and fatty acyl CoA as substrates. (ymdb.ca)
Enzymes4
  • CerS enzymes have different affinities for acyl-CoA substrates, resulting in the generation of dihydroceramides with differing chain lengths (C14-C26). (frontiersin.org)
  • a) Enzymes that catalyse the removal of hy-drogen from a substrate but use only oxy-gen as a hydrogen acceptor to form water as a reaction product (with the exception of uricase and monoamine oxidase which form H 2 O 2 ). (biologydiscussion.com)
  • Despite recent progresses in uncovering the biology underlying age-related diseases, and improvements in research focused on the molecular mechanisms of age-related diseases, our knowledge of the cellular mechanisms that regulate key mitochondrial energy substrate enzymes in the aging heart is greatly underdeveloped. (wvu.edu)
  • Within the Krebs cycle acetyl-CoA, combined with oxaloacetate, undergoes cycles involving eight enzymes, in which reducing equivalents are produced which then enter the electron transfer chain for the production of energy as adenosine triphosphate (ATP). (oncohemakey.com)
Biosynthesis1
  • Although differing in substrate specificity, subcellular localization, and tissue distribution, all isozymes of this family convert free long-chain fatty acids into fatty acyl-CoA esters, and thereby play a key role in lipid biosynthesis and fatty acid degradation. (thermofisher.com)
Mitochondrial energy1
  • Impact of short- and medium-chain organic acids, acylcarnitines, and acyl-CoAs on mitochondrial energy metabolism. (medscape.com)
Mitochondria2
  • Acetyl-CoA enters the Krebs cycle, also known as the citric acid or tricarboxylic (TCA) cycle, within the mitochondria. (oncohemakey.com)
  • 18 - 21 The liver mitochondria convert additional acetyl-CoA into ketone bodies beta-hydroxybutyrate (BOHB), acetoacetate (AcAc), and acetate (Ac) or oxalacetate. (jomes.org)
Specificity1
Thioester2
  • Catalyzes the initial reaction in intramitochondrial fatty acid synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. (joplink.net)
  • Together, these results suggest that fibrates exhibit markedly different abilities to alter the extent of covalent binding of 2-PPA to hepatic protein by differentially modulating the hepatic exposure of the two reactive metabolites of 2-PPA, namely 2-PPA-CoA thioester and acyl glucuronide. (aspetjournals.org)
Vitro1
Dehydrogenation1
  • C6-C10-dicarboxylic aciduria: investigations of a patient with riboflavin responsive multiple acyl-CoA dehydrogenation defects. (medscape.com)
Lipid synthesis2
  • Lipid synthesis requires bulk carbon skeleton acyl-CoAs, the transport of which depends on the acyl-CoA binding protein (ACBP). (biomedcentral.com)
  • Fatty acids provide the acyl skeleton for lipid synthesis. (biomedcentral.com)
Synthesis1
  • Fatty acid (FA) profile assays revealed that HpDGAT2A, HpDGAT2D, and HpDGAT2E, but not HpDGAT2B, preferred monounsaturated fatty acyl-CoAs (MUFAs) for TAG synthesis in yeast cells, and showed a preference for polyunsaturated fatty acyl-CoAs (PUFAs) based on their feeding strategy. (researchsquare.com)
Moiety3
  • Cleavage of the 3-keto compound at the now unstable alpha-beta carbon bond and transfer of another CoA moiety to the new fragment results in 2 products: acetyl-CoA, composed of the carbonyl and original alpha-carbon from the starting molecule, and a new fatty acyl-CoA that is 2 carbons shorter than the original molecule. (medscape.com)
  • The arrangement between the flavin ring and deprotonated 3S-C8-CoA is consistent with a charge transfer interaction with the negatively charged acyl-chain of 3S-C8-CoA as an electron donor stacking on the pyrimidine moiety of the flavin ring as an electron acceptor. (rcsb.org)
  • The bound CoA molecule has its β-mercaptoethanolamine moiety extended into the active site with the terminal SH group close to active center Cys291, enabling formation of the reaction intermediate by acylation of Cys291. (afpm.org.my)
Ketone bodies1
Electron1
  • The structure of the highest occupied molecular orbital of the complex revealed the electron flow pathway from a substrate to the flavin ring. (rcsb.org)
Residues3
  • SET domain protein methyltransferases catalyze the transfer of methyl groups from the cofactor S -adenosylmethionine (AdoMet) to specific lysine residues of protein substrates, such as the N-terminal tails of histones H3 and H4 and the large subunit of the Rubisco holoenzyme complex. (nature.com)
  • We have identified a cytosolic acyl-CoA binding protein, with highly conserved amino acid residues and a typical acyl-CoA binding domain in N. caninum . (biomedcentral.com)
  • Here we define the precise Rab29 binding region of the LRRK2 Armadillo domain between residues 360-450 and show that this domain, termed 'Site #1', can also bind additional LRRK2 substrates, Rab8A and Rab10. (stanford.edu)
Reaction2
  • This complex can simulate the metastable reaction intermediate immediately after the alpha-proton elimination of a substrate and before the beta-hydrogen transfer as a hydride, and is therefore regarded as a transition-state analog. (rcsb.org)
  • Therefore, the amount of substrate can be determined by the c hromogenic development of the oxidative coupling reaction. (whdsbio.cn)
Arachidonate2
  • Preferentially uses arachidonate and eicosapentaenoate as substrates. (nih.gov)
  • This isozyme preferentially utilizes arachidonate as substrate. (nih.gov)
Protein3
  • In contrast, significant decreases of the hepatic levels of 2-PPA acyl glucuronide and/or 2-PPA-CoA by fenofibrate and gemfibrozil significantly lowered the covalent binding of 2-PPA to hepatic protein. (aspetjournals.org)
  • Lipoate protein ligase B primarily recognizes the C8-phosphopantetheine arm of its donor substrate and weakly binds the acyl carrier protein. (du.ac.in)
  • Obviously, enhanced dietary protein intake has the same net effect by flooding the mitochondrion with propionyl CoA. (medscape.com)
MCAD1
  • The crystalline complex was obtained by co-crystallizing MCAD in the oxidized form with 3S-C8-CoA. (rcsb.org)
Phosphorylation1
Amino acid1
  • Other sources of propionyl CoA include odd chain-length fatty acids and the side chain of cholesterol, although these probably contribute very little in relation to the amino acid sources. (medscape.com)
Cholesterol2
  • As propionyl-CoA is a product of cholesterol catabolism, we propose that NAT could have a role in the utilization of this important cofactor. (ox.ac.uk)
  • Glucose, alcohol, acyl-CoA and cholesterol can be used to detect those substrates coupled with the new Trinder 's reagent and 4-AA. (whdsbio.cn)
Accumulation1
  • Accumulation of the 3-carbon fatty acyl-CoA within the mitochondrion leads to decreased free CoA for other reactions, which is alleviated by conversion of propionyl CoA to propionyl-carnitine. (medscape.com)
Defects1
  • Subsequent work led to further delineation of another disorder, initially called multiple carboxylase deficiency, which includes deficiency of propionyl CoA carboxylase activity in addition to defects in other carboxylases. (medscape.com)
Molecule2
  • Once inside the mitochondrion, the action of CPT II at the inner surface of the membrane releases free carnitine, which exits to the cytosol and leaves behind the acyl-CoA molecule. (medscape.com)
  • The next step is the introduction of a water molecule and resaturation of the double bond to form fatty enoyl-CoA. (medscape.com)
Pathophysiology1
  • Its pathophysiology is likely to involve insulin resistance at the level of both skeletal muscle and visceral adipose tissue and altered fluxes of metabolic substrates between these tissues that in turn impair liver metabolism. (erudit.org)
Insulin1
  • It is believed that theproduction of ceramide can reduce insulin efficiency via long chain acyl-CoA. (heraldopenaccess.us)
Efficiency1
  • The ETC is organized into inner mitochondrial membrane supercomplexes that promote substrate channeling and catalytic efficiency. (oroboros.at)
Upstream1
  • ACSL4 regulates components of the two complexes of the mTOR pathway (mTORC1/2), along with upstream regulators and substrates. (oncotarget.com)
Reactions1
  • the mitochondrial CoA, thus freed, can participate in other reactions or once again become involved in formation of propionyl CoA. (medscape.com)
Metabolism1
  • The formation of propionyl CoA in human metabolism is derived from many sources, chiefly catabolism of a number of essential amino acids (isoleucine, valine, threonine, methionine). (medscape.com)
Catalyze1
  • a) They catalyze the removal of hydrogen from a substrate but not able to use oxy-gen as hydrogen acceptor. (biologydiscussion.com)
Catabolism1
  • In this study we identify, in Arabidopsis, an oxalyl-CoA decarboxylase (AtOXC) that is capable of catalyzing the second step in this proposed pathway of oxalate catabolism. (usda.gov)
Preference1
  • May have some preference toward very-long-chain substrates. (joplink.net)
Excess1
  • Acetyl-CoA in excess of requirements for energy production via the Krebs cycle is converted via lipogenesis to stored lipids in adipocytes. (oncohemakey.com)
Oxygen1
  • a) They catalyse the removal of hydrogen from a substrate and use either oxygen or artificial substances such a methylene blue as hydrogen acceptor. (biologydiscussion.com)
Activity1
  • The specific activity of TBNAT was tested against a broad range of acyl-CoA cofactors using hydralazine as a substrate. (ox.ac.uk)
Product1
  • The open conformation is stabilized by the asymmetric dimerization and enables PhaC to accommodate CoA and also to create the product egress path. (afpm.org.my)
Free3
  • Instead, they can use a free acyl-CoA substrate for chain elongation. (wikipedia.org)
  • Dietary fats, and lipids produced endogenously from acetyl-CoA, are initially hydrolysed by lipases into glycerol and free fatty acids. (oncohemakey.com)
  • The structure reveals an asymmetric dimer, in which one protomer adopts an open conformation bound to CoA, whereas the other adopts a closed conformation in a CoA-free form. (afpm.org.my)
Backbone2
  • These are glycerolipids lipids containing a common glycerol backbone to which at least one fatty acyl group is esterified. (hmdb.ca)
  • TG(10:0/15:0/20:1(13Z)) belongs to the family of triradyglycerols, which are glycerolipids lipids containing a common glycerol backbone to which at least one fatty acyl group is esterified. (ymdb.ca)
Transfer1
  • The large stabilization energy by charge transfer probably plays an important role in determining the alignment of the flavin ring with 3S-C8-CoA. (rcsb.org)
Mechanisms1
  • Current trends in the supplementation and fitness industries have led to the use of ergogenic aids to increase exercise performance, substrate utilization, and the mechanisms behind these factors. (jomes.org)