pyruvate dehydrogenase complex


pyruvate dehydrogenase complex
a multienzyme complex consisting of multimers of the three distinct enzymes: pyruvate dehydrogenase (acetyl-transferring) [EC 1.2.4.1], dihydrolipoyllysine-residue acetyltransferase [EC 2.3.1.12], and dihydrolipoyl 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.

Medical dictionary. 2011.

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