| 2-methylacetoacetyl-CoA thiolase | <enzyme> An enzyme that is part of the l-isoleucine degradation pathway; it catalyses the conversion of 2-methylacetoacetyl-CoA (CH3COCH(CH3)COSCoA) to acetyl-CoA and propionyl-CoA. A deficiency of this enzyme leads to an accumulation of 2-methylacetoacetyl-CoA, causing episodes of severe metabolic acidosis and ketosis. (05 Mar 2000) |
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| 2-methylacyl-CoA dehydrogenase | <enzyme> Involved in isoleucine and valine metabolism; from rat liver mitochondria Registry number: EC 1.3.99.12 Synonym: s-2-methylbutyryl-CoA dehydrogenase, isobutyryl-CoA dehydrogenase, 2-methyl-branched chain acyl coenzyme a dehydrogenase, 2-methyl-branched chain acyl-CoA dehydrogenase, 2-methylacyl-coenzyme a dehydrogenase, branched-chain acyl-CoA dehydrogenase (26 Jun 1999) |
| 2-methyl branched-chain enoyl-CoA reductase | <enzyme> Important in the reversal of beta-oxidation under anaerobic conditions in ascaris suum Registry number: EC 1.3.1.52 Synonym: 2-methyl bce-CoA reductase (26 Jun 1999) |
| 2-oxo-delta(3)-4,5,5--trimethylcyclopentenyl acetyl-CoA monooxygenase | <enzyme> Nadph-dependent baeyer-villiger monooxygenase that catalyses conversion of 2-(2'-acetoxyethyl)cyclohexanone to 7-(2'-acetoxyethyl)-2-oxepanone Registry number: EC 1.14.13.- Synonym: odtac monooxygenase (26 Jun 1999) |
| 2-oxo-delta-(3)-4,5,5-trimethylcyclopentenylacetyl-CoA oxygenase | <enzyme> Specific for NADPH as electron donor; incorporates single molecule of oxygen Registry number: EC 1.14.13.- Synonym: otmcpa CoA oxygenase, 2-oxo-delta-(3)-4,5,5-trimethylcyclopentenylacetyl-coenzyme a oxygenase (26 Jun 1999) |
| 3-aminobutyryl-CoA deaminase | <enzyme> Forms crotonyl CoA Registry number: EC 4.3.1.- Synonym: 3-aminobutyryl-coenzyme a deaminase (26 Jun 1999) |
| 3-hydroxy-2-methylpropionyl-CoA dehydrogenase | <enzyme> Forms methylmalonyl-CoA saemialdehyde Registry number: EC 1.1.1.- Synonym: 3-hydroxyisobutyryl-CoA dehydrogenase (26 Jun 1999) |
| 3-hydroxy-3-methylglutaryl-CoA | -OOCCH2C(OH)(CH3)CH2COS-CoA;a key intermediate in the synthesis of ketone bodies and of steroids. Synonym: 3-hydroxy-3-methylglutaryl-CoA. Beta-hydroxy-beta-methylglutaryl-lyase, an enzyme, found primarily in liver and rumen epithelium that catalyses the formation of acetyl-CoA and acetoacetate from beta-hydroxy-beta-methylglutaryl-CoA; a key step in ketogenesis; a deficiency of this enzyme leads to episodes of severe metabolic acidosis without ketosis. Beta-hydroxy-beta-methylglutaryl-reductase, an enzyme that catalyses the rate-limiting step of cholesterol biosynthesis, beta-hydroxy-beta-methylglutaryl-CoA + 2NADPH + 2H+ → mevalonate + 2NADP+ + coenzyme A. Beta-hydroxy-beta-methylglutaryl-synthase, an enzyme in mitochondria that catalyses the reaction of acetyl-CoA with acetoacetyl-CoA and water to form beta-hydroxy-beta-methylglutaryl-CoA and coenzyme A, a step required for both ketogenesis and steroidogenesis to occur. (05 Mar 2000) |
| 3-hydroxyacyl-CoA dehydrogenase | Beta-Hydroxyacyl dehydrogenase;enzyme catalyzing the oxidation of an l-3-hydroxyacyl-CoA to a 3-ketoacyl-CoA with reduction of NAD+; one of the enzymes of the b oxidation of fatty acids. Synonym: beta-ketohydrogenase, beta-ketoreductase. (05 Mar 2000) |
| 3-hydroxyacyl CoA dehydrogenases | <enzyme> Enzymes that reversibly catalyze the oxidation of a 3-hydroxyacyl CoA to 3-ketoacyl CoA in the presence of NAD. They are key enzymes in the oxidation of fatty acids and in mitochondrial fatty acid synthesis. Chemical name: (S)-3-Hydroxyacyl-CoA:NAD+ oxidoreductase Registry number: EC 1.1.1.35 (12 Dec 1998) |
| 3-ketoacid-CoA transferase | <enzyme> An enzyme catalyzing the reversible conversion of acetoacetyl-CoA and succinate into succinyl-CoA and acetoacetate; malonyl-CoA can substitute for succinyl-CoA and a few other 3-oxo acids for the acetoacetate; an important step in order for the ketone bodies to serve as a fuel for extrahepatic tissues. Synonym: 3-ketoacid-CoA transferase, acetoacetyl-succinic thiophorase. (05 Mar 2000) |
| 3-ketoacyl-CoA thiolase | <enzyme> An enzyme catalyzing the thioclastic cleavage by coenzyme A of beta-ketoacyl-CoA, forming an acyl-CoA with a carbon chain shorter by two atoms, the missing two atoms appearing as acetyl-CoA. A step in fatty acid degradation. See: acetyl-CoA acetyltransferase. Synonym: 3-ketoacyl-CoA thiolase, beta-ketothiolase. (05 Mar 2000) |
| 3-methylcrotonyl-CoA | (CH3)2C==CHCOSCoA;an intermediate in the degradation of l-leucine; accumulates in a deficiency of 3-methylcrotonyl-CoA carboxylase. 3-methylcrotonyl-CoA carboxylase, an enzyme in the pathway of l-leucine degradation that catalyses the reaction of 3-methylcrotonyl-CoA with CO2, ATP, and water to form ADP, orthophosphate, and 3-methylglutaconyl-CoA; a deficiency of this enzyme causes episodes of severe metabolic acidosis. (05 Mar 2000) |
| 3-methylglutaconyl-CoA hydratase | <enzyme> An enzyme that catalyses the reaction of trans-3-methylglutaconyl-CoA and water to form 3-hydroxy-3-methylglutaconyl-CoA; this enzyme participates in the pathway for l-leucine degradation; a deficiency of this enzyme will result in 3-methylglutaconic aciduria. (05 Mar 2000) |
| 3-oxoacid-CoA transferase | <enzyme> An enzyme catalyzing the reversible conversion of acetoacetyl-CoA and succinate into succinyl-CoA and acetoacetate; malonyl-CoA can substitute for succinyl-CoA and a few other 3-oxo acids for the acetoacetate; an important step in order for the ketone bodies to serve as a fuel for extrahepatic tissues. Synonym: 3-ketoacid-CoA transferase, acetoacetyl-succinic thiophorase. (05 Mar 2000) |