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ALME acetyl-lysine methyl ester
ALTEE acetyl-L-tyrosine ethyl ester
ATEE N-acetyl-1-tyrosyl-ethyl ester
ATH acetyl-tyrosine hydrazide
LAAM L-alpha acetyl methadol
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CoA Coarctation of the Aorta
FAO Fatty acyl CoA oxidase
HMGR HMG CoA reductase
IVD Isovaleryl-CoA dehydrogenase
LCHAD Long chain 3-hydroxyacyl-CoA-dehydrogenase
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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)
malonyl-CoA The condensation product of malonic acid and coenzyme A, an intermediate in fatty acid biosynthesis.
<chemical> S-malonylcoenzyme A. A coenzyme a derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.
Chemical name: Coenzyme A, S-(hydrogen propanedioate)
Synonym: malonylcoenzyme A.
(05 Mar 2000)
malonyl-CoA-3,4-dichloroaniline N-malonyltransferase <enzyme> From peanuts
Registry number: EC 2.3.1.-
Synonym: 3,4-dichloroaniline n-malonyltransferase, dca malonyltransferase, malonyl-coenzyme a-3,4-dichloroaniline n-malonyltransferase
(26 Jun 1999)
malonyl-CoA-anthranilic acid N-malonyltransferase <enzyme> From peanuts
Registry number: EC 2.3.1.-
Synonym: anthranilic acid n-malonyltransferase, aa malonyltransferase, malonyl-coenzyme a-anthranilic acid n-malonyltransferase
(26 Jun 1999)
malonyl-CoA-D-tryptophan N-malonyltransferase <enzyme> From peanuts
Registry number: EC 2.3.1.-
Synonym: trp-malonyltransferase, malonyl-coenzyme a-d-tryptophan n-malonyltransferase
(26 Jun 1999)
malonyl-CoA-isoflavone-7-O-glucoside-6''-O-malonyltransferase <enzyme> From chick pea roots; specificity for isoflavones formononetin and biochanin a
Registry number: EC 2.3.1.-
Synonym: mcoa-if7g malonyltransferase, malonyl-coenzyme a-isoflavone-7-o-glucoside-6''-o-malonyltransferase
(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)
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