| 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) |
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| 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 carboxylase |
[EC 6.4.1.3] an enzyme of the ligase class that catalyzes the carboxylation of propionyl CoA to form methylmalonyl CoA; the reaction is part of the route by which three-carbon compounds from some amino acids and from odd numbered fatty acids are used as fuels. The enzyme is an oligomer comprising 4α and 4β chains and requires a biotin cofactor. Deficiency of enzyme activity due to a defect in either chain, an autosomal recessive trait, causes propionicacidemia; see also multiple carboxylase deficiency, under carboxylase.
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