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and catalyzes the reoxidation of the dihydrolipoyl prostheticgroup attached to the lysyl residue(s) of the acyltransferasecomponents of the three α-keto acid dehydrogenase com-plexes and to the hydrogen-carrier protein of the glycinecleavage system. Because E3 is a common component in thethree α-keto acid dehydrogenases, a decrease in E3 activitycan affect the activities of all three complexes, which canresult in increased urinary excretion of α-keto acids, elevatedblood lactate, pyruvate and branched chain amino acids. E3 belongs to the pyridine nucleotide-disulfide oxidore-ductase family along with glutathione reductase, thioredoxinreductase, mercuric reductase and trypanothione reductase.

Characterization of Three Site-specific Mutations in Human Dihydrolipoamide Dehydrogenase (Gly-42 to Ala, Gly-43 to Ala and Val-48 to Trp)