• 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)
  • 3-hydroxyacyl-CoA dehydrogenase/ enoyl-CoA hydratase [Arabidopsis thaliana] >gi6728. (cornell.edu)
  • Dehydrogenation: Catalyzed by 3-hydroxyacyl-CoA dehydrogenase, this step generates NADH. (assaygenie.com)
  • The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. (nih.gov)
  • Roles of hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha, a lipid metabolism enzyme, in Wilms tumor patients. (nih.gov)
  • 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)
  • 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)
  • 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)
  • 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)
  • Peroxisomal fatty acid beta-oxidation multifunctional protein [Oryza sativa (japonica cu. (cornell.edu)
  • This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. (nih.gov)
  • 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. (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 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)
  • 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)
  • A mitochondrial protein consisting of four alpha-subunits and four beta-subunits. (bvsalud.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 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)
  • 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)
  • Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain acyl CoA dehydrogenase deficiency (LCHAD) deficiency. (medscape.com)
  • Beta-oxidation primarily takes place within the mitochondria, a specialized component of the cell known as the powerhouse. (assaygenie.com)
  • During this process, long-chain acyl-CoA molecules, the main components of fatty acids, are broken down into acetyl-CoA molecules. (assaygenie.com)
  • Here, fatty acids are activated for degradation by conjugation with coenzyme A (CoA) in the cytosol. (assaygenie.com)
  • Beta-oxidation is a multi-step process that involves the breakdown of fatty acids within the body. (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)
  • 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)
  • 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)
  • Lipids as an energy source - TAG degradation in cells, β-oxidation of fatty acids. (wikilectures.eu)
  • 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)
  • 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)
  • 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)
  • 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)
  • Merged oxidation reaction down to a C18 with a set degradation pathway. (umassmed.edu)
  • What is the β-oxidation pathway? (flashcardmachine.com)
  • Dehydrogenation: Catalyzed by acyl-CoA dehydrogenase, this step removes two hydrogens between carbons 2 and 3. (assaygenie.com)
  • LCHAD deficiency is a severe fatty acid oxidation disorder that is fatal if untreated. (medscape.com)
  • Role of short-chain hydroxyacyl CoA dehydrogenases in SCHAD deficiency. (ox.ac.uk)
  • Crystallization and preliminary X-ray analysis of (R)-specific enoyl-CoA hydratase from Aeromonas caviae involved in polyhydroxyalkanoate biosynthesis" (PDF). (wikipedia.org)
  • Futile cycling of intermediates of fatty acid biosynthesis toward peroxisomal beta-oxidation in Saccharomyces cerevisiae. (unil.ch)
  • Metabolic reprogramming as a crucial emerging hallmark of cancer is critical for tumor cells to maintain cellular bioenergetics, biosynthesis and reduction/oxidation (REDOX) balance. (oncotarget.com)
  • They contribute to many crucial metabolic processes such as fatty acid oxidation, biosynthesis of ether lipids and free radical detoxification. (genome.jp)
  • 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)
  • BbsCD represents a novel clade of related enzymes within the SDR family, which adopt a heterotetrameric architecture and catalyze the β-oxidation of aromatic succinate adducts. (nih.gov)
  • Enzymes catalyzing the so-called ω- and α-oxidation occur on the membranes of the endoplasmic reticulum . (wikilectures.eu)
  • HP15_2693 (GFF2749) is involved in phenylalanine degradation via phenylacetyl-CoA and likely has this activity. (lbl.gov)
  • This enzyme catalyses the following chemical reaction on D-3-hydroxyacyl-CoA This enzyme catalyses a hydration step in peroxisomal beta oxidation. (wikipedia.org)
  • Hydration: Catalyzed by enoyl-CoA hydratase, this step adds water across the double bond. (assaygenie.com)
  • 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)
  • 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)
  • 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)
  • 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)
  • β-oxidation - breaking down MK into acetyl~CoA, which enters the Krebs cycle , or ketone bodies are formed from it. (wikilectures.eu)
  • Variants in the ethylmalonyl-CoA decarboxylase (ECHDC1) gene: a novel player in ethylmalonic aciduria? (nih.gov)
  • β-oxidation taking place in the mitochondrial matrix is ​​of major importance. (wikilectures.eu)
  • Enoyl-CoA hydratase 2 (2-enoyl-CoA hydratase 2, AtECH2, ECH2, MaoC, MFE-2, PhaJAc, D-3-hydroxyacyl-CoA hydro-lyase, D-specific 2-trans-enoyl-CoA hydratase) is an enzyme (EC 4.2.1.119) with systematic name (3R)-3-hydroxyacyl-CoA hydro-lyase. (wikipedia.org)
  • Predicted to enable carboxy-lyase activity and enoyl-CoA hydratase activity. (nih.gov)
  • ATP-citrate lyase/succinyl-CoA ligase [Interproscan]. (ntu.edu.sg)
  • citramalyl-CoA lyase [Source:HGNC Symbo. (gsea-msigdb.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)
  • Predicted to be involved in fatty acid beta-oxidation. (nih.gov)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • This Hydrogenophaga strain also expressed genes indicative of chemolithoautotrophy, including CO 2 fixation, H 2 oxidation, S-compound oxidation, and denitrification. (frontiersin.org)
  • The genes for the alpha and beta subunits have been localized to chromosome 2. (medscape.com)
  • By comparison, we found that various genes associated with acyl editing, fatty acid β-oxidation, triacylglycerol assembly and oil-body formation had greater expression levels at middle developmental stage (38 DAP), which are consistent with the fast accumulation of EFA in V. galamensis developing seed, implying their fundamental roles in EFA production. (researchsquare.com)
  • We found that ligand activation of PPARγ dramatically induced de novo lipid synthesis as well as fatty acid beta (β)-oxidation in lung cancer both in vitro and in vivo . (oncotarget.com)
  • 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)
  • Steady-state kinetic constants for HADH 1 and HADH 2 suggest that type 1 is the major enzyme involved in mitochondrial beta-oxidation of short-chain hydroxyacyl-CoAs. (ox.ac.uk)