• ATP generation occurs through beta-oxidation of fat and glucose oxidation through glycolysis, both of which lead to acetyl CoA production. (veteriankey.com)
  • 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)
  • Cell Rep. … Once in the matrix, carnitine palmitoyltransferase II (CPT-II) hydrolyses the acyl-carnitine to free carnitine and LC-CoA, for beta-oxidation. (siderac.com)
  • and in the human diacylglycerol acyltransferase 1 and 2 assays. (sigmaaldrich.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)
  • 3a,7a-dihydroxy-5b-cholestanoyl-CoA remains intact while alpha-methylacyl-CoA racemase moves it along through the peroxisome. (smpdb.ca)
  • The citric acid cycle is a complex of many enzymes that convert the by-products of glucose, protein, and fat degradation as pyruvate (glucose and some amino acids) and acetate (fatty acids and some amino acids) into covalently bound CoA moieties to make acetyl CoA, the major entry point substrate for the citric acid cycle ( Figure 4.1 ). (veteriankey.com)
  • Figure 4.1 The citric acid cycle showing acetyl CoA entry and the formation of NADH and FADH 2 with the liberation of CO 2 and GTP. (veteriankey.com)
  • This process is catalyzed by the enzyme acetyl-CoA synthetase. (medscape.com)
  • Decanoyl coenzyme A (Decanoyl CoA) is coupled with S-adenosylmethionine (SAM) by Vibrio cholera CqsA enzyme to produce a potent quorum-sensing molecule, 3-aminotridec-2-en-4-one (Ea-CAI-1. (sigmaaldrich.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)
  • His Endocrinology research includes themes of Enzyme assay, Enzyme, Newborn screening, Myopathy and Acyl CoA dehydrogenase. (research.com)
  • An enzyme that catalyzes the transfer of acetyl groups from ACETYL-COA to arylamines. (wakehealth.edu)
  • An enzyme that catalyzes reversibly the conversion of palmitoyl-CoA to palmitoylcarnitine in the inner mitochondrial membrane. (lookformedical.com)
  • Carnitine palmitoyltransferase 1 (CPT1) is the enzyme in the outer mitochondrial membrane that converts long-chain acyl-CoA species to their corresponding long-chain acyl-carnitines for transport into the mitochondria (see Fig. Long‐Term Follow‐Up of Bezafibrate Treatment in Patients With the Myopathic Form of Carnitine Palmitoyltransferase 2 Deficiency. (siderac.com)
  • CPT-2 deficiency occurs when an enzyme, called carnitine palmitoyl transferase - type 2 (CPT-2… Carnitine palmitoyltransferase 2 (CPT-2 or CPT-II) is one of a family of carnitine acyltransferases in humans that catalyze the reversible transfer of acyl groups between coenzyme A (CoASH) and L-carnitine, converting fatty acyl-CoA esters into fatty acyl-carnitine esters. (siderac.com)
  • To assess the effects of acylcarnitine accumulation on muscle insulin sensitivity, a model of muscle acylcarnitine accumulation was generated by deleting carnitine palmitoyltransferase 2 (CPT2) specifically from skeletal muscle (Cpt2 Sk−/− mice).CPT2 is an irreplaceable enzyme for mitochondrial long-chain fatty acid oxidation, converting matrix acylcarnitines to acyl-CoAs. (siderac.com)
  • With the help of water, peroxisomal multifunctional enzyme type 2 turns 3a,7a-dihydoxy-5b-cholest-24-enoyl-CoA into 3a,7a,24-trihydoxy-5b-cholestanoyl-CoA. (smpdb.ca)
  • This compound then uses peroxisomal multifunctional enzyme type 2 to create chenodeoxycholoyl-CoA. (smpdb.ca)
  • Carnitine palmitoyl transferase II deficiency, myopathic form Disease definition The myopathic form of carnitine palmitoyltransferase II (CPT II) deficiency, an inherited metabolic disorder that affects mitochondrial oxidation of long chain fatty acids (LCFA), is the most common and the least severe form of CPT II deficiency (see this term). (siderac.com)
  • Baicalin, as a flavonoid glycoside, is an allosteric carnitine palmityl transferase 1 ( CPT1 ) activator. (medchemexpress.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)
  • Longer-term (24 and 48 h) treatment reduced the expression of lipogenic markers (FA synthase and sterol regulatory element-binding protein-1) and increased the expression of lipolytic [hormone-sensitive lipase (HSL) and adipose triglyceride lipase (ATGL)] and mitochondrial (peroxisome proliferator-activated receptor γ coactivator-1α and carnitine palmitoyltransferase 1) markers. (academic-accelerator.com)
  • The mitochondrial membrane‐associated carnitine palmitoyltransferase system is a validated target for the treatment of type 2 diabetes mellitus. (siderac.com)
  • The inhibition by malonyl-CoA is characteristic of carnitine palmitoyltransferase type 1, but not of type 2. (siderac.com)
  • Carnitine O-palmitoyltransferase 1, muscle isoform (EC: 2.3.1.21 Search proteins in UniProtKB for this EC number. (siderac.com)
  • Carnitine palmitoyltransferase 2 (CPT2) deficiency: This rare genetic condition prevents the body from using certain fats for energy. (siderac.com)
  • A novel mutation leading to the lethal form of carnitine palmitoyltransferase type-2 deficiency. (siderac.com)
  • Carnitine palmitoyltransferase (CPT) deficiency is a group of disorders caused by defects in the enzymes CPT I or II. (siderac.com)
  • Carnitine palmitoyltransferase II deficiency is an inherited disorder of mitochondrial long-chain fatty acid oxidation. (siderac.com)
  • Carnitine palmitoyltransferase 2 (CPT2) deficiency is a condition that prevents the body from using certain fats for energy, particularly during periods without food (fasting). (siderac.com)
  • Carnitine palmitoyltransferase 1A (CPT1A) deficiency is a disorder of fatty acid oxidation, the process by which the body breaks down fatty acids from food for energy. (siderac.com)
  • From there, propionyl-CoA and chenodeoxycholoyl-CoA join forces and enlist the help of non-specific lipid transfer protein to further chenodeoxycholoyl-CoAâ€TMs journey in the peroxisome. (smpdb.ca)
  • Revisiting 7a-hydroxy-cholestene-3-one, the second chain of reactions it is involved in follows a similar path as the first, moving through the mitochondria, endoplasmic reticulum and peroxisome until choloyl-CoA is formed, which then is used in three reactions so that its derivatives may leave the cell to interact with intestinal microflora and become taurodeoxycholic acid, deoxycholic acid glycine conjugate and deoxycholic acid. (smpdb.ca)
  • Fatty esters include important biochemical intermediates such as wax esters , fatty acyl thioester coenzyme A derivatives, fatty acyl thioester ACP derivatives and fatty acyl carnitines. (wikidoc.org)
  • Carnitine acyltransferase (CAT) transports free fatty acids into the mitochondria and therefore regulates their entry into the oxidative pathway. (medscape.com)
  • Acetic acid (an acyl group carrier) is linked with coenzyme A (a thiol) to produce Acetyl-CoA. (medscape.com)
  • Decanoyl coenzyme A is a substrate for acyltransferase. (sigmaaldrich.com)
  • It can also catalyze acetyl transfer between arylamines without COENZYME A and has a wide specificity for aromatic amines, including SEROTONIN. (wakehealth.edu)
  • Peroxisomal acyl coenzyme A oxidase 2 converts 3a,7a-dihydroxy-5b-cholestanoyl-CoA into 3a,7a-dihydoxy-5b-cholest-24-enoyl-CoA. (smpdb.ca)
  • Supports fat burning by increasing carnitine biosynthesis (molecule required for mitochondrial fatty acid oxidation) (97,98). (ultra18body.com)
  • Regulated by malonyl CoA. (flashcardmachine.com)
  • Fatty acyls (including fatty acids ) are a diverse group of molecules synthesized by chain-elongation of an acetyl-CoA primer with malonyl-CoA or methylmalonyl-CoA groups. (wikidoc.org)
  • Palmityl-CoA is a strong basic compound (based on its pKa). (ymdb.ca)
  • DNA microarray expression profiling indicated that dietary administration of NDGA upregulated the expression of certain genes involved in fatty acid oxidation and their transcription regulator, PPARα, decreased the expression of a number of lipogenic genes and relevant transcription factors, and differentially impacted the genes of fatty acid transporters, acetyl CoA synthetases, elongases, fatty acid desaturases and lipid clearance proteins in liver, skeletal muscle and adipose tissues. (biomedcentral.com)
  • Palmityl-CoA, also known as palmitoyl CoA or CoA, palmitoyl, belongs to the class of organic compounds known as long-chain fatty acyl coas. (ymdb.ca)
  • Acetyl-CoA is the acyl-CoA of acetic acid (also known as acetate). (amboss.com)
  • The membrane of peroxisomes in Saccharomyces cerevisiae is impermeable to NAD(H) and acetyl-CoA under in vivo conditions. (research.com)
  • Sphingolipids are a complex family of compounds [7] that share a common structural feature, a sphingoid base backbone that is synthesized de novo from serine and a long-chain fatty acyl CoA, then converted into ceramides , phosphosphingolipids, glycosphingolipids and other species. (wikidoc.org)
  • Then, bile acyl-CoA synthetase teams up with 3a,7a-dihydroxycoprostanic acid to create 3a,7a-dihydroxy-5b-cholestanoyl-CoA. (smpdb.ca)
  • β-oxidation - breaking down MK into acetyl~CoA, which enters the Krebs cycle , or ketone bodies are formed from it. (wikilectures.eu)
  • It catalyzes the transfer of long-chain fatty acids, preferentially unsaturated fatty acids, to lysophosphatides with the formation of 1,2-diacylglycero-3-phosphocholine and CoA. (wakehealth.edu)