| 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) |
| clofibroyl-CoA hydrolase | <enzyme> Rat liver cytosolic enzyme is not the same as palmitoyl CoA hydrolase (EC 3.1.2.2) Registry number: EC 3.1.2.- Synonym: clofibroyl-coenzyme a hydrolase (26 Jun 1999) |
| CoA | <abbreviation> Coenzyme A. (05 Mar 2000) |
| CoA transferases | Thiaphorases;enzymes transferring CoA from acetyl-CoA or succinyl-CoA to other acyl radicals. (05 Mar 2000) |
| phytanoyl-CoA ligase | <enzyme> An acyl-CoA ligase found in peroxisomes of fibroblasts; it is distinct from palmitoyl-CoA ligase and lignoceroyl-CoA ligase Registry number: EC 6.2.1.- Synonym: phytanoyl-coenzyme a ligase (26 Jun 1999) |
| cyclohexylideneacetyl-CoA hydratase | <enzyme> From arthrobacter; forms (1-hydroxycyclohexan-1-yl)acetyl-CoA Registry number: EC 4.2.1.- Synonym: ca-CoA-hydratase, cyclohexylideneacetyl-coenzyme a hydratase (26 Jun 1999) |
| pristanoyl-CoA oxidase | <enzyme> Pristanoic acid undergoes beta-oxidation giving alternatively rise to acetyl-CoA or propionyl-CoA and h2o2 Registry number: EC 1.3.3.- Synonym: 2,6,10,14-tetramethylpentadecanoyl-CoA oxidase, 2-methylpalmitoyl-CoA oxidase, pristanyl-CoA oxidase (26 Jun 1999) |
| propionyl-CoA | The coenzyme A thioester derivative of propionic acid; an intermediate in the degradation of l-valine, l-isoleucine, l-threonine, l-methionine, and odd-chain fatty acids; a precursor for the synthesis of odd-chain fatty acids; it accumulates in individuals with a deficiency of propionyl-CoA carboxylase. Propionyl-CoA carboxylase, an enzyme that catalyses the reaction of propionyl-CoA with CO2 and ATP to produce ADP, inorganic phosphate, and d-methylmalonyl-CoA; a biotin-dependent enzyme; an inherited deficiency of this enzyme will lead to propionic acidemia and developmental retardation. (05 Mar 2000) |
| propionyl-CoA carboxylase | <enzyme> See also propionyl CoA carboxylase (ATP-hydrolyzing) (EC 6.4.1.4) Registry number: EC 4.1.1.41 Synonym: methylmalonyl-CoA decarboxylase, propionyl-coenzyme a carboxylase (26 Jun 1999) |
| propionyl CoA hydrolase | <enzyme> Nadh sensitive; found in brown adipose tissue mitochondria of rat; prevents a deterimental increase of propionyl-CoA in mitochondria; mw at least 240 kD Registry number: EC 3.1.2.- Synonym: propionyl coenzyme a hydrolase (26 Jun 1999) |