| 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) |
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| 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) |
| 3-oxobutyryl-CoA reductase | <enzyme> Nadh-dependent, (s)-specific enzyme from clostridium tyrobutyricum; n-terminal amino acid sequence given in first source Registry number: EC 1.1.1.- (26 Jun 1999) |
| 4-halobenzoate-CoA ligase | <enzyme> Involved in conversion of 4-chlorobenzoate to 4-hydroxybenzoate by pseudomonas sp. Cbs3; 115 kD; acts on 4-chloro-, 4-bromo-, 4-iodobenzoate most effectively; 16 n-terminal amino acid sequence determined, with 3 questions, in first source Registry number: EC 6.2.1.- Synonym: 4-chlorobenzoate-CoA ligase, 4-cba-CoA ligase, 4-chlorobenzoate-coenzyme a ligase (26 Jun 1999) |
| 4-hydroxybenzoyl-CoA hydrolase | <enzyme> Final enzyme in the degradation of 4-chlorobenzoic acid by pseudomonas; liberates 4-hydroxybenzoic acid; amino acid sequence has been determined Registry number: EC 3.1.2.- Synonym: 4-hydroxybenzoyl-coenzyme a thioesterase, 4-hba-CoA thioesterase (26 Jun 1999) |
| 4-hydroxybutyrate CoA-transferase | <enzyme> Forms 4-hydroxybutyryl-CoA from 4-hydroxybutyrate and acetyl-CoA Registry number: EC 2.8.3.- Synonym: 4-hydroxybutyrate coenzyme a transferase (26 Jun 1999) |
| 4-hydroxybutyryl-CoA dehydratase | <enzyme> Forms vinylacetyl-CoA from 4-hydroxybutyryl-CoA Registry number: EC 4.2.1.- Synonym: gamma-hydroxybutyryl-CoA dehydratase (26 Jun 1999) |
| 5-hydroxyvalerate CoA-transferase | <enzyme> Catalyses the formation of 5-hydroxyvaleryl-CoA from 5-hydroxyvalerate and acetyl-CoA; also acts on propionate and 5-hydroxy-2-pentenoate Registry number: EC 2.8.3.- (26 Jun 1999) |
| 5-hydroxyvaleryl-CoA dehydratase | <enzyme> Contains fad; bifunctional enzyme which catalyses reversible dehydration of 5-hydroxyvaleryl-CoA to 4-pentenoyl-CoA Registry number: EC 4.2.1.- Synonym: 5hvc-dehydratase, 5-hydroxyvaleryl-CoA dehydratase-dehydrogenase (26 Jun 1999) |
| lignoceroyl-CoA ligase | <enzyme> Requires ATP and mg Registry number: EC 6.2.1.- Synonym: lignoceroyl-coenzyme a ligase (26 Jun 1999) |
| lignoceroyl-CoA oxidase | <enzyme> Catalyses the beta-oxidation of lignoceroyl-CoA Registry number: EC 1.3.3.- (26 Jun 1999) |
| long-chain 3-hydroxyacyl CoA dehydrogenase | <enzyme> Deficiency may cause some cases of hypoglycaemic 3-hydroxydicarboxylic aciduria Registry number: EC 1.1.1.211 Synonym: l-chad (26 Jun 1999) |
| long-chain-acyl-CoA dehydrogenase | <enzyme> Flavoprotein; forms with another flavoprotein plus EC 1.5.5.1 a system reducing ubiquinone and other acceptors Registry number: EC 1.3.99.- Synonym: lc-acyl-CoA dehydrogenase, long-chain-acyl-coenzyme a dehydrogenase, very-long-chain acyl-CoA dehydrogenase, vlcad (26 Jun 1999) |
| long-chain-fatty-acid-CoA ligase | <enzyme> Fatty acid thiokinase (long-chain), a ligase forming acyl-CoA, AMP, and pyrophosphate from long-chain fatty acids, ATP, and coenzyme A. Activity is independent of phosphatidylcholine Registry number: EC 6.2.1.3 Synonym: acyl-activating enzyme, dodecanoyl-CoA synthetase, fatty acid thiokinase (long chain), acid-coenzyme a ligase, fatty acid-CoA ligase, acyl-CoA synthetase, acyl-CoA ligase, coash ligase, ciprofibroyl-CoA synthetase, pristanoyl-CoA synthetase, palmityl CoA synthetase, palmitoyl CoA synthetase, palmitoyl CoA ligase, fatty acyl-CoA synthetase, very long chain fatty acid acyl-CoA synthetase, vlcfa acyl-CoA synthetase, nafenopin-CoA ligase, palmitoyl-CoA synthase, faa2 gene product, faa2p protein, vlacs enzyme (26 Jun 1999) |