pyruvate dehydrogenase (acetyl-transferring)

pyruvate dehydrogenase (acetyl-transferring)
py·ru·vate de·hy·dro·gen·ase (ac·e·tyl-trans·fer·ring) (piґroo-vāt de-hiґdro-jən-ās asґə-təl transґfər-ing) [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 the lipoamide linked to the side chain of a lysine residue of the enzyme dihydrolipoyllysine-residue acetyltransferase, which is also part of the complex. Deficiency of the enzyme causes lacticacidemia, ataxia, psychomotor retardation, and sometimes lactic acidosis.

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