pyruvate dehydrogenase complex


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py·ru·vate de·hy·dro·gen·ase

a structurally distinct collection of enzymes containing pyruvate dehydrogenase (lipoamide), dihydrolipoyl transacetylase, and dihydrolipoyl dehydrogenase.

pyruvate dehydrogenase complex

[pī′ro̅o̅·vāt dē·hī′drō·jən·ās]
a multienzyme complex consisting of at least three distinct enzymes that catalyzes the formation of acetyl coenzyme A from pyruvate and coenzyme A. The acetyl coenzyme A is used in fatty acid synthesis, for acetylations, and for oxidation by means of the citric acid cycle. Deficiency of any component of the complex results in excess of lactic acid in the blood, ataxia, and psychomotor retardation.
References in periodicals archive ?
Evidence for existence of tissue-specific regulation of the mammalian pyruvate dehydrogenase complex.
Mutations in the X-linked pyruvate dehydrogenase (E1) [alpha] subunit gene (PDHA1) in patients with a pyruvate dehydrogenase complex deficiency.