| pyruvate dehydrogenase (lipoamide) |
[EC 1.2.4.1] an enzyme of the oxidoreductase class that is a component of the multienzyme pyruvate dehydrogenase complex (q.v.). The enzyme catalyzes the oxidative decarboxylation of pyruvate, forming acetyl bound to the cofactor thiamine pyrophosphate; the acetyl is subsequently transferred to lipoamide to form acetyldihydrolipoamide, an intermediate in the overall reaction catalyzed by the complex. Deficiency of the enzyme causes lacticacidemia, ataxia, psychomotor retardation, and sometimes lactic acidosis.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
|---|---|
| pyruvate dehydrogenase c. |
a multienzyme complex consisting of at least three distinct enzymes: pyruvate dehydrogenase (lipoamide) [EC 1.2.4.1], dihydrolipoamide S-acetyltransferase [EC 2.3.1.12], and dihydrolipoamide dehydrogenase [EC 1.8.1.4]. The integrated enzyme complex requires the cofactors thiamine pyrophosphate, lipoic acid, coenzyme A, FAD, and NAD+. It catalyzes the formation of acetyl coenzyme A from pyruvate and coenzyme A, using NAD+ as an electron acceptor; the acetyl coenzyme A is used in fatty acid synthesis, for acetylations, and for oxidation via the tricarboxylic acid cycle. Deficiency of any component of the complex results in lacticacidemia, ataxia, and psychomotor retardation. See also lipoamide dehydrogenase deficiency.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|
| pyruvate dehydrogenase complex |
see under complex.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|