| hydroxymethylbilane synthase |
[EC 4.3.1.8] an enzyme of the lyase class that catalyzes the deamination and condensation of four molecules of porphobilinogen to form a linear tetrapyrrole intermediate, hydroxymethylbilane, which cyclizes to form uroporphyrinogen. Alone, the enzyme produces uroporphyrinogen I; in the presence of uroporphyrinogen synthase it produces uroporphyrinogen III, the physiologically important isomer. Deficiency of the enzyme, an autosomal dominant trait, results in acute intermittent porphyria. Called also porphobilinogen deaminase and uroporphyrinogen I synthase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
|---|---|
| hydroxymethylglutaryl-CoA lyase |
[EC 4.1.3.4] an enzyme of the lyase class that catalyzes the cleavage of 3-hydroxy-3-methylglutaryl CoA to form acetyl coenzyme A and acetoacetate. The reaction is a step in ketogenesis and in the catabolism of leucine. Deficiency of the enzyme, an autosomal recessive trait, causes 3-hydroxy-3-methylglutaricaciduria.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
| hydroxymethylglutaryl-CoA reductase (NADPH) |
[EC 1.1.1.34] an enzyme of the oxidoreductase class that catalyzes the reduction of 3-hydroxy-3-methylglutaryl CoA to mevalonate, using NADPH as an electron donor. The reaction is a key rate-limiting step in the biosynthesis of cholesterol and the enzyme is inactivated by a specific kinase and reactivated by a specific phosphorylase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
| hydroxymethylglutaryl-CoA synthase |
[EC 4.1.3.5] an enzyme of the lyase class that catalyzes the condensation of acetyl coenzyme A and acetoacetyl coenzyme A to form 3-hydroxy-3-methylglutaryl CoA. A liver and kidney mitochondrial enzyme catalyzes the reaction as a step in ketogenesis; a cytosolic enzyme acts in the synthesis of cholesterol and other isoprenoids.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
| hydroxymethyltransferase |
a term used in the names of some of the enzymes of the sub-subclass hydroxymethyl-, formyl-, and related transferases [EC 2.1.2] to denote those that catalyze the transfer of a hydroxymethyl group from a donor to an acceptor compound. Cf. formyltransferase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|