| butyryl-CoA dehydrogenase | <enzyme> Electron-transferring flavoprotein system reducing ubiquinone and other acceptors; formerly EC 1.3.2.1; a short-chain acyl-CoA dehydrogenase Registry number: EC 1.3.99.2 Synonym: butyryl dehydrogenase, butyryl-coenzyme a dehydrogenase (26 Jun 1999) |
|---|---|
| butyryl-CoA synthetase | Fatty acid thiokinase (medium chain), a ligase forming acyl-CoA's from medium-chain fatty acids and CoA with the conversion of ATP to AMP and PPi. A key step in activation of fatty acids. Synonym: acyl-activating enzyme, butyryl-CoA synthetase, octanoyl-CoA synthetase. (05 Mar 2000) |
| caffeoyl-CoA O-methyltransferase | <enzyme> Catalyses the methylation of caffeoyl-CoA to form trans-feruloyl-CoA; also accepts with low affinity various other caffeic esters as substrates; do not confuse with EC 2.1.1.68 which acts on caffeic acid; has been sequenced; genbank l22203 (stellaria longipes) Registry number: EC 2.1.1.104 Synonym: sam-c-CoA 3-o-methyltransferase, s-adenosylmethionin-trans-caffeoyl-coenzyme a 3-o-methyltransferase, sam-cca-methyltransferase, caffeoyl-CoA-3-o-methyltransferase, s-adenosyl-l-methionine-trans-caffeoyl-coenzyme a 3-o-methyltransferase, ccoaomt, s-adenosylmethionine-trans-caffeoyl-CoA 3-o-methyltransferase, ccoamt (26 Jun 1999) |
| palmitoyl-CoA dependent dehydrogenase | <chemical> Sum of reactions catalyzed by fatty acyl-CoA oxidase, enoyl-CoA hydratase (EC 4.2.1.17) and beta-hydroxyacyl-CoA dehydrogenase (EC 1.1.1.35) Synonym: palmitoyl-coenzyme a dependent dehydrogenase (26 Jun 1999) |
| palmitoyl-CoA hydrolase | <enzyme> Enzyme catalyzing reversibly the hydrolysis of palmitoyl-CoA or other long-chain acyl coenzyme a compounds to yield CoA and palmitate or other acyl esters. The enzyme is involved in the esterification of fatty acids to form triglycerides. Chemical name: Palmitoyl-CoA hydrolase Registry number: EC 3.1.2.2 (12 Dec 1998) |
| palmitoyl CoA oxidase | <enzyme> In peroxisomes; forms hydrogen peroxide Registry number: EC 1.3.3.- Synonym: palmityl CoA oxidase, palmitoyl coenzyme a oxidase (26 Jun 1999) |
| glutaconyl CoA decarboxylase | <enzyme> Forms crotonyl CoA Registry number: EC 4.1.1.- Synonym: glutaconyl coenzyme a decarboxylase (26 Jun 1999) |
| glutaryl-CoA | The mono thiol ester of coenzyme A and glutaric acid; an intermediate in l-lysine and l-tryptophan catabolism. Glutaryl-CoA dehydrogenase, an enzyme that catalyses the reaction of glutaryl-CoA with an acceptor to form crotonoyl-CoA, CO2, and the reduced acceptor; a deficiency of this enzyme will lead to either glutaric acidemia type I or hyperoxaluria type II. Glutaryl-CoA synthetase, an enzyme similar to acyl-CoA synthetase, but which splits ATP, GTP, or ITP to the nucleoside diphosphate and orthophosphate in acting on glutarate, thus forming glutaryl-Coa. (05 Mar 2000) |
| glutaryl-CoA dehydrogenase | <enzyme> Glutaric aciduria may be due to deficiency of this enzyme Registry number: EC 1.3.99.7 Synonym: glutaryl CoA dehydrogenase, glutaryl-coenzyme a dehydrogenase (26 Jun 1999) |
| medium-chain acyl-CoA dehydrogenase | <enzyme> Deficiency in mitochondrial beta oxidation of fatty acids gives a disorder frequently masquerading as reye syndrome or sudden infant death; leads to buildup of octanoyl carnitine in urine Registry number: EC 1.3.- Synonym: mcaca-dehydrogenase, medium-chain acyl-coenzyme a dehydrogenase, octanoyl-CoA dehydrogenase, mcad enzyme (26 Jun 1999) |
| methylglutaconyl-CoA hydratase | <enzyme> Catalyses conversion of 3-hydroxy-3-methylglutaryl-CoA to trans-3-methylglutaconyl-CoA and water Registry number: EC 4.2.1.18 Synonym: 3-hydroxy-3-methylglutaryl-CoA hydro-lyase, 3-methylglutaconyl-coenzyme a hydratase, 3-methylglutaconyl-CoA hydratase (26 Jun 1999) |
| methylmalonyl-CoA | An intermediate in the degradation of several metabolites (e.g., valine, methionine, odd-chain fatty acids, theronine); elevated in cases of pernicious anaemia. (05 Mar 2000) |
| methylmalonyl-CoA epimerase | <enzyme> An enzyme that catalyses the interconversion of d-methylmalonyl-CoA and l-methylmalonyl-CoA. (05 Mar 2000) |
| methylmalonyl-CoA mutase | <enzyme> An enzyme that catalyses the conversion of methylmalonyl-CoA to succinyl-CoA by transfer of the carbonyl group. It requires a cobamide coenzyme. A block in this enzymatic conversion leads to the metabolic disease, methylmalonic aciduria. Chemical name: (R)-2-Methyl-3-oxopropanoyl-CoA CoA-carbonylmutase Registry number: EC 5.4.99.2 (12 Dec 1998) |
| phenylacetate - CoA ligase | <enzyme> Catalyses the synthesis of phenylacetyl-CoA and phenoxyacetyl-CoA Registry number: EC 6.2.1.30 Synonym: phenylacetyl-CoA ligase, phenylacetic acid activating enzyme, phenoxyacetic acid activating enzyme, phenacyl coenzyme a ligase, phenylacetate-CoA ligase (AMP-forming) (26 Jun 1999) |