Characterization of Two Site-specific Mutations in Human Dihydrolipoamide Dehydrogenase

摘要

Dihydrolipoamide dehydrogenase (E3) (dihydrolipoamide: NAD~+ oxidoreductase; EC 1.8.1.4) is a homodimeric flavo-enzyme as shown in Figure S1. E3 catalyzes the reoxidation of the dihydrolipoyl prosthetic group attached to the lysyl residue(s) of the acyltransferase components of three α-keto acid dehydrogenase complexes (pyruvate, α-ketoglutarate and branched-chain α-keto acid dehydrogenase complexes). E3 along with glutathione reductase, thioredoxin reductase, mercuric reductase and trypanothione reductase belongs to the pyridine nucleotide-disulfide oxidoreductase family.