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Enoyl-CoA HydrataseFatty AcidsPeroxisomesOxidation-ReductionFumarate HydrataseEnoyl-(Acyl-Carrier-Protein) Reductase (NADH)Hydro-LyasesLipid MetabolismLiverTriclosan3-Hydroxyacyl CoA DehydrogenasesAcyl Coenzyme ALeiomyomatosisOxidoreductasesCoenzyme APeroxisomal Bifunctional EnzymeRhodococcusDodecenoyl-CoA Isomerase3-Oxoacyl-(Acyl-Carrier-Protein) ReductaseOxidoreductases Acting on CH-CH Group DonorsFatty Acid SynthasesFatty Acid DesaturasesInterleukin-1betaCarbon-Carbon Double Bond IsomerasesFatty Acid Synthesis InhibitorsKineticsMolecular Sequence DataPeroxisomal Multifunctional Protein-2Enoyl-CoA Hydratase 2BrevibacteriumRacemases and EpimerasesFatty Acid Synthase, Type IIIsomerasesMicrobodiesSubstrate SpecificityEnoyl-(Acyl-Carrier Protein) Reductase (NADPH, B-Specific)Amino Acid Sequencebeta 2-MicroglobulinStereoisomerismFatty Acid Synthase, Type IMitochondrial Trifunctional ProteinMultienzyme ComplexesTungstenPhenyl EthersCrotonatesEimeria tenellaCoenzyme A LigasesMolecular StructureNADBase SequenceReceptors, Adrenergic, betaAeromonas caviaeModels, MolecularIntegrin beta3Binding SitesCrystallography, X-RayNADPAconitate HydrataseThaueraCatalysisEstersChromatography, High Pressure LiquidBacterial ProteinsAcrylonitrileAcetyl Coenzyme ASequence Homology, Amino AcidRecombinant ProteinsProtein ConformationCloning, MolecularTransforming Growth Factor betaEscherichia coliMitochondria, LiverStructure-Activity RelationshipEpoxide HydrolasesPseudomonasCoenzyme A-TransferasesDeltaproteobacteriaHydrogen-Ion ConcentrationMalatesAcyl-CoA OxidasePolyhydroxyalkanoatesHydrogen PeroxideIsocitrates17-Hydroxysteroid DehydrogenasesCysteineGlutaratesGlucoseCells, CulturedCitratesNeoplastic Syndromes, HereditaryMutationRNA, MessengerIntegrin alpha5beta1Hydroxymethylglutaryl CoA ReductasesIntegrin beta4Citric Acid CycleMalonyl Coenzyme AAnaerobiosisPseudomonas putidaEpoxy Compounds
IsomeraseHydroxyacyl-CoA dehydrogenaseProteinPathwayLong-chain acyl CoA dehydrogeMitochondriaIntermediatesSubunitSynthetaseGeneMitochondrial matrixFatty acid metabReactionHydrationSevere fatty acid oxiDeficiencyDehydrogenasesMoleculeKetone bodiesCoenzymeLyaseInvolved in fattyECHS1NADHDegradationComplete oxidationLCHADCarnitineCarbonsMetabolismCytosolThioesterBiosynthesisReactionsSource:HGNCAlpha and beta subunits
Isomerase1
  • The gene product is a member of the hydratase/isomerase superfamily. (wikipedia.org)
Hydroxyacyl-CoA dehydrogenase11
  • The N-terminal region of the encoded protein contains enoyl-CoA hydratase activity while the C-terminal region contains 3-hydroxyacyl-CoA dehydrogenase activity. (utsouthwestern.edu)
  • Long-chain hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency and trifunctional protein (TFP) deficiency are caused by impairment of mitochondrial TFP. (nih.gov)
  • TFP has three enzymatic activities - long-chain enoyl-CoA hydratase, long-chain 3-hydroxyacyl-CoA dehydrogenase, and long-chain 3-ketoacyl-CoA thiolase. (nih.gov)
  • In individuals with LCHAD deficiency, there is isolated deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase, while deficiency of all three enzymes occurs in individuals with TFP deficiency. (nih.gov)
  • The mitochondrial trifunctional protein, composed of 4 alpha and 4 beta subunits, catalyzes 3 steps in mitochondrial beta-oxidation of fatty acids: long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), long-chain enoyl-CoA hydratase, and long-chain thiolase activities. (nih.gov)
  • Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that make up the trifunctional protein of the inner mitochondrial membrane. (medscape.com)
  • Short-chain hydroxyacyl CoA dehydrogenase deficiency is an ill-defined, severe pediatric disorder of mitochondrial fatty acid beta-oxidation of short-chain hydroxyacyl CoAs. (ox.ac.uk)
  • [4] Trifunctional protein deficiency is characterized by decreased activity of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), long-chain enoyl-CoA hydratase, and long-chain thiolase. (wikidoc.org)
  • HP15_1512 (GFF1550) is annotated as enoyl-CoA hydratase but likely has 3-hydroxyacyl-CoA dehydrogenase activity as well. (lbl.gov)
  • It contains enoyl-CoA hydratase, long-chain-3-hydroxyacyl-CoA dehydrogenase, and acetyl-CoA C-acyltransferase activities and plays an important role in the metabolism of long chain FATTY ACIDS. (bvsalud.org)
  • Dehydrogenation: Catalyzed by 3-hydroxyacyl-CoA dehydrogenase, this step generates NADH. (assaygenie.com)
Protein18
  • The protein encoded by this gene functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. (wikipedia.org)
  • Enzyme & Protein. (wikipedia.org)
  • The protein encoded by this gene is a bifunctional enzyme and is one of the four enzymes of the peroxisomal beta-oxidation pathway. (utsouthwestern.edu)
  • Trifunctional protein deficiency is characterized by decreased activity of all 3 enzymes. (nih.gov)
  • 2003). Genetic Heterogeneity of Mitochondrial Trifunctional Protein Deficiency See also MTPD2 (620300), caused by mutation in the HADHB gene, the beta subunit of the mitochondrial trifunctional protein. (nih.gov)
  • The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase (LCKT). (medscape.com)
  • The protein is an octamer composed of 4 alpha subunits that contain the LCEH and LCHAD activities and 4 beta subunits that contain the LCKT activity. (medscape.com)
  • The etiology of the severe peripheral neuropathy of trifunctional protein deficiency may result from the unique metabolite, 3-keto-acyl-CoA, after conversion to a methylketone via spontaneous decarboxylation. (medscape.com)
  • The protein encoded by this gene is a bifunctional enzyme that is involved in the peroxisomal beta-oxidation pathway for fatty acids. (nih.gov)
  • Defects in this gene that affect the peroxisomal fatty acid beta-oxidation activity are a cause of D-bifunctional protein deficiency (DBPD). (nih.gov)
  • This gene encodes the beta subunit of the mitochondrial trifunctional protein, a catalyst of mitochondrial beta-oxidation of long chain fatty acids . (wikidoc.org)
  • The HADHB protein catalyzes the final step of beta-oxidation, in which 3-ketoacyl CoA is cleaved by the thiol group of another molecule of Coenzyme A . The thiol is inserted between C-2 and C-3, which yields an acetyl CoA molecule and an acyl CoA molecule, which is two carbons shorter. (wikidoc.org)
  • The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. (wikidoc.org)
  • GapMind searches the predicted proteins for candidates by using ublast (a fast alternative to protein BLAST) to find similarities to characterized proteins or by using HMMer to find similarities to enzyme models (usually from TIGRFams ). (lbl.gov)
  • Candidates for each step are identified by using ublast (a fast alternative to protein BLAST) against a database of manually-curated proteins (most of which are experimentally characterized) or by using HMMer with enzyme models (usually from TIGRFam ). (lbl.gov)
  • A mitochondrial protein consisting of four alpha-subunits and four beta-subunits. (bvsalud.org)
  • The protein is an octamer composed of 4 alpha subunits that contain the LCEH and long-chain 3-hydroxy acyl-coenzyme A dehydrogenase activities, and 4 beta subunits that contain the LCKT activity. (medscape.com)
  • specific protein associated with the reaction, generic enzyme associated with the reaction, and the reaction itself. (umassmed.edu)
Pathway8
  • Enoyl-CoA hydratase (ECH) catalyzes the second step in beta-oxidation pathway of fatty acid metabolism. (wikipedia.org)
  • This study characterizes two enzymes of this pathway, (E)-benzylidenesuccinyl-CoA hydratase (BbsH), and (S,R)-2-(α-hydroxybenzyl)succinyl-CoA dehydrogenase (BbsCD) from Thauera aromatica. (nih.gov)
  • Merged oxidation reaction down to a C18 with a set degradation pathway. (umassmed.edu)
  • What is the β-oxidation pathway? (flashcardmachine.com)
  • The shortened acyl-CoA then reenters the beta-oxidation pathway. (assaygenie.com)
  • Acetyl-CoA, generated by the beta-oxidation pathway, enters the mitochondrial TCA cycle, where it is further oxidized to generate NADH and FADH2. (assaygenie.com)
  • In conclusion, the fatty acid beta-oxidation pathway is a key component of our body's metabolic machinery, enabling efficient energy production and storage. (assaygenie.com)
  • For example, in T cells, beta-oxidation is crucial for differentiation and effector functions, with different T cell subsets (e.g., effector T cells and memory T cells) showing varying dependencies on this metabolic pathway. (assaygenie.com)
Long-chain acyl CoA dehydroge1
  • Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain acyl CoA dehydrogenase deficiency (LCHAD) deficiency. (medscape.com)
Mitochondria1
  • Beta-oxidation primarily takes place within the mitochondria, a specialized component of the cell known as the powerhouse. (assaygenie.com)
Intermediates4
  • It catalyzes the hydration of 2-trans-enoyl-coenzyme A (CoA) intermediates to L-3-hydroxyacyl-CoAs. (wikipedia.org)
  • However, this effect may be achieved at the cost of fatty acid infiltration and of toxic effects of β-oxidation intermediates on organ functions. (medscape.com)
  • It also acts as a catalyst for the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids. (nih.gov)
  • Futile cycling of intermediates of fatty acid biosynthesis toward peroxisomal beta-oxidation in Saccharomyces cerevisiae. (unil.ch)
Subunit2
  • Trifunctional enzyme subunit beta, mitochondrial (TP-beta) also known as 3-ketoacyl-CoA thiolase , acetyl-CoA acyltransferase , or beta-ketothiolase is an enzyme that in humans is encoded by the HADHB gene . (wikidoc.org)
  • ATP synthase F1 subunit beta [Source:HG. (gsea-msigdb.org)
Synthetase1
Gene3
  • Variants in the ethylmalonyl-CoA decarboxylase (ECHDC1) gene: a novel player in ethylmalonic aciduria? (nih.gov)
  • Gene sets follow the order of enzymes. (umassmed.edu)
  • Not available " indicates that no score was available for the corresponding gene, enzyme, or reaction in the relevant database. (umassmed.edu)
Mitochondrial matrix1
  • β-oxidation taking place in the mitochondrial matrix is ​​of major importance. (wikilectures.eu)
Fatty acid metab1
Reaction2
  • At the beginning of this reaction, 1 molecule of 'H2O', and 1 molecule of 'trans-Oct-2-enoyl-CoA' are present. (reactome.org)
  • At the end of this reaction, 1 molecule of '(S)-Hydroxyoctanoyl-CoA' is present. (reactome.org)
Hydration1
Severe fatty acid oxi1
  • LCHAD deficiency is a severe fatty acid oxidation disorder that is fatal if untreated. (medscape.com)
Deficiency1
  • Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency. (ox.ac.uk)
Dehydrogenases3
  • BbsH, a member of the enoyl-CoA hydratase family, converts (E)-benzylidenesuccinyl-CoA to 2-(α-hydroxybenzyl)succinyl-CoA and was subsequently used in a coupled enzyme assay with BbsCD, which belongs to the short-chain dehydrogenases/reductase (SDR) family. (nih.gov)
  • To understand the relative contributions of the two known short-chain hydroxyacyl CoA dehydrogenases (HADH) tissue biopsies of six distinct family individuals were analyzed and kinetic parameters were compared. (ox.ac.uk)
  • Comment: This step is described by 1.1.1.35, a broader term for 3-hydroxyacyl-CoA dehydrogenases. (lbl.gov)
Molecule3
  • Thus, 3-oxopalmitoyl-CoA is considered to be a fatty ester lipid molecule. (ymdb.ca)
  • 3-oxopalmitoyl-CoA is a very hydrophobic molecule, practically insoluble (in water), and relatively neutral. (ymdb.ca)
  • Remarkably, the complete oxidation of one palmitate molecule (a fatty acid containing 16 carbons) generates 129 ATP molecules, showcasing the efficiency of this process. (assaygenie.com)
Ketone bodies2
  • Thus, the clinical features may result from either toxicity due to long-chain acyl-CoA esters that cause cardiomyopathy and cardiac arrhythmias or from a block in long-chain fatty acid oxidation that leads to an inability to synthesize ketone bodies and/or adenosine triphosphate from long-chain fatty acids. (medscape.com)
  • β-oxidation - breaking down MK into acetyl~CoA, which enters the Krebs cycle , or ketone bodies are formed from it. (wikilectures.eu)
Coenzyme1
Lyase3
Involved in fatty1
  • Predicted to be involved in fatty acid beta-oxidation. (nih.gov)
ECHS11
  • Enoyl-CoA hydratase short chain has been confirmed to interact with STAT3, such that ECHS1 specifically represses STAT3 activity by inhibiting STAT3 phosphorylation. (wikipedia.org)
NADH1
  • Both NADH and FADH2 are produced by both beta-oxidation and the TCA cycle and are used by the mitochondrial electron transport chain to produce ATP . (assaygenie.com)
Degradation3
  • Anaerobic toluene degradation proceeds by fumarate addition to produce (R)-benzylsuccinate as first intermediate, which is further degraded via β-oxidation by five enzymes encoded in the conserved bbs operon. (nih.gov)
  • Lipids as an energy source - TAG degradation in cells, β-oxidation of fatty acids. (wikilectures.eu)
  • HP15_2693 (GFF2749) is involved in phenylalanine degradation via phenylacetyl-CoA and likely has this activity. (lbl.gov)
Complete oxidation1
  • The complete oxidation of 1 g of TAG yields approximately 38 kJ , while only 17 kJ are obtained from 1 g of carbohydrates or proteins. (wikilectures.eu)
LCHAD1
  • This enzyme complex metabolizes long-chain fatty acids, and LCHAD activity is specific for compounds of C12-C16 chain length. (medscape.com)
Carnitine1
  • The resulting long-chain fatty-acyl-CoA is then modified by carnitine palmitoyltransferase 1 (CPT1) to acylcarnitine, which is then transported across the inner mitochondrial membrane by carnitine translocase (CAT). (assaygenie.com)
Carbons3
  • Fatty acids represent a rich source of energy for gluconeogenesis , but glucose is not formed from their carbon atoms (with the exception of fatty acids with an odd number of C). Acetyl-CoA cannot be converted to either pyruvate or oxaloacetate - both carbons are split off as CO 2 during the Krebs cycle . (wikilectures.eu)
  • Dehydrogenation: Catalyzed by acyl-CoA dehydrogenase, this step removes two hydrogens between carbons 2 and 3. (assaygenie.com)
  • Thiolytic cleavage: Catalyzed by beta-ketothiolase, this step cleaves the terminal acetyl-CoA group and forms a new acyl-CoA which is two carbons shorter than the previous one. (assaygenie.com)
Metabolism2
  • Beta-oxidation plays an integral role not only in energy metabolism but also in immune function. (assaygenie.com)
  • These cells alter their metabolism in response to changes in the immune environment, and beta-oxidation of fatty acids is one way they generate the necessary energy and biosynthetic precursors for their function. (assaygenie.com)
Cytosol1
  • This process is governed by the ER acetylation machinery: the cytosol:ER-lumen acetyl-CoA transporter AT-1 (also known as SLC33A1), and the ER-resident lysine acetyltransferases ATase1 and ATase2 (also known as NAT8B and NAT8, respectively). (biologists.com)
Thioester1
  • The enzyme is involved in the formation of a β-hydroxyacyl-CoA thioester. (wikipedia.org)
Biosynthesis1
  • They contribute to many crucial metabolic processes such as fatty acid oxidation, biosynthesis of ether lipids and free radical detoxification. (genome.jp)
Reactions2
  • Individual types of fatty acid oxidation are indicated by Greek letters, which determine the carbon atom on which the reactions take place. (wikilectures.eu)
  • trans-2-hexenoyl-CoA = 28 reactions were found. (brenda-enzymes.org)
Source:HGNC4
Alpha and beta subunits1
  • The genes for the alpha and beta subunits have been localized to chromosome 2. (medscape.com)