• 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)
  • 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)
  • [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)
  • Dehydrogenation: Catalyzed by 3-hydroxyacyl-CoA dehydrogenase, this step generates NADH. (assaygenie.com)
  • 3-hydroxyacyl-CoA dehydrogenase PaaC (EC 1.1.1. (lbl.gov)
  • 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)
  • The protein encoded by this gene functions in the second step of the mitochondrial fatty acid beta-oxidation pathway. (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)
  • 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)
  • 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 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)
  • Some patients who are deficient in all 3 enzymatic activities of the protein have been described, although most have an isolated LCHAD deficiency, which results in the inability to metabolize long-chain fatty acids. (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 incidence of isolated LCHAD activity deficiency and trifunctional protein deficiency is unknown in the United States. (medscape.com)
  • HADHB is a subunit of the mitochondrial trifunctional protein and has thiolase activity. (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)
  • A mitochondrial protein consisting of four alpha-subunits and four beta-subunits. (bvsalud.org)
  • Long-chain 3-hydroxy acyl-coenzyme A dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that make up the trifunctional protein of the inner mitochondrial membrane. (medscape.com)
  • 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)
  • Occurrence frequency of either isolated long-chain 3-hydroxy acyl-coenzyme A dehydrogenase activity deficiency or trifunctional protein deficiency is unknown in the United States. (medscape.com)
  • Enoyl-(Acyl carrier protein) reductase [Interproscan]. (ntu.edu.sg)
  • 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)
  • During this process, long-chain acyl-CoA molecules, the main components of fatty acids, are broken down into acetyl-CoA molecules. (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)
  • 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)
  • 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)
  • 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)
  • β-oxidation - breaking down MK into acetyl~CoA, which enters the Krebs cycle , or ketone bodies are formed from it. (wikilectures.eu)
  • 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)
  • Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain acyl CoA dehydrogenase deficiency (LCHAD) deficiency. (medscape.com)
  • 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)
  • CPT2 reconverts the long-chain acylcarnitine back to long-chain acyl-CoA before beta-oxidation. (assaygenie.com)
  • Dehydrogenation: Catalyzed by acyl-CoA dehydrogenase, this step removes two hydrogens between carbons 2 and 3. (assaygenie.com)
  • The shortened acyl-CoA then reenters the beta-oxidation pathway. (assaygenie.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)
  • Patients with long-chain 3-hydroxy acyl-coenzyme A dehydrogenase activity deficiency usually present with hypoketotic hypoglycemia, cardiomyopathy, hypotonia, and hepatomegaly at a median age of 6 months. (medscape.com)
  • Acyl CoA Synthetase: What is it/What does it do? (flashcardmachine.com)
  • What happens if you lack the proteins to transport Acyl-Coa into the matrix? (flashcardmachine.com)
  • acyl-CoA thioesterase 13 [Source:HGNC S. (gsea-msigdb.org)
  • Variants in the ethylmalonyl-CoA decarboxylase (ECHDC1) gene: a novel player in ethylmalonic aciduria? (nih.gov)
  • The gene product is a member of the hydratase/isomerase superfamily. (wikipedia.org)
  • METHODS: We investigated clinical, genetic (variants in SLC22A5 gene) and functional (carnitine transport activity in fibroblasts) characteristics of all referred individuals through NBS (newborns and mothers) and clinically diagnosed patients with PCD (not through NBS). (bvsalud.org)
  • Enoyl-CoA hydratase (ECH) catalyzes the second step in beta-oxidation pathway of fatty acid metabolism. (wikipedia.org)
  • 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)
  • What is the β-oxidation pathway? (flashcardmachine.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)
  • This enzyme complex metabolizes long-chain fatty acids, and LCHAD activity is specific for compounds of C12-C16 chain length. (medscape.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)
  • Lipids as an energy source - TAG degradation in cells, β-oxidation of fatty acids. (wikilectures.eu)
  • The enzyme is involved in the formation of a β-hydroxyacyl-CoA thioester. (wikipedia.org)
  • 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)
  • 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)
  • LCHAD deficiency is a severe fatty acid oxidation disorder that is fatal if untreated. (medscape.com)
  • Predicted to be involved in fatty acid beta-oxidation. (nih.gov)
  • 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)
  • 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)
  • Carnitine transport activity completely separated severe phenotype from benign phenotype (median (range): 4.0% (3.5-5.0)] vs 26% (9.5-42.5), respectively). (bvsalud.org)
  • Genetic variants and carnitine transport activity can be used to distinguish between these groups. (bvsalud.org)
  • HP15_2693 (GFF2749) is involved in phenylalanine degradation via phenylacetyl-CoA and likely has this activity. (lbl.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)
  • 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)
  • DNA microarray analyses using these cell lines were performed with or without adding each subtype ligand and provided much important information on the PPAR target genes involved in lipid metabolism, transport, storage and other activities. (biomedcentral.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)
  • The genes for the alpha and beta subunits have been localized to chromosome 2. (medscape.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)
  • Chemoautotrophic activity in the dark ocean has been suggested to be on a similar order of magnitude as heterotrophic activity and thus might contribute considerably to carbon and energy recycling in the ocean's interior [ 3 ]. (biomedcentral.com)
  • Enzymes catalyzing the so-called ω- and α-oxidation occur on the membranes of the endoplasmic reticulum . (wikilectures.eu)
  • Immune cells, like macrophages, T cells , and B cells , rely on metabolic pathways to fuel their activities, and beta-oxidation is one such key metabolic process. (assaygenie.com)
  • This Hydrogenophaga strain also expressed genes indicative of chemolithoautotrophy, including CO 2 fixation, H 2 oxidation, S-compound oxidation, and denitrification. (frontiersin.org)
  • Information is collected on the next exams пїЅ MammaPrint: A genomic take a look at that analyzes the activity of certain genes in early-stage breast cancer. (ehd.org)
  • Comment: This step is described by 1.1.1.35, a broader term for 3-hydroxyacyl-CoA dehydrogenases. (lbl.gov)
  • 3-oxopalmitoyl-CoA, also known as 3-oxohexadecanoyl-CoA, belongs to the class of organic compounds known as long-chain 3-oxoacyl coas. (ymdb.ca)
  • Consistent with this relatively high divergence among the subtype-specific ligand binding domains, differential activation of PPARs by endogenous and exogenous compounds may account for the specific biological activity of the three PPAR subtypes [ 2 , 3 ]. (biomedcentral.com)