Dihydrolipoamide dehydrogenase

The dihydrolipoyl dehydrogenase ( dldH, E3, Protein L ) is the enzyme in all living organisms, which catalyzes the dehydrogenation of dihydrolipoamide. As E3 subunit of pyruvate dehydrogenase complex (PDC) and α -ketoglutarate dehydrogenase complex is essential for the maintenance of the glycolysis and the TCA cycle. As a subunit of the E3 - Branched α -keto acids dehydrogenase complex ( BCKD ), it is necessary to break down the amino acids valine, leucine and isoleucine. Moreover dldH part of the glycine -cleaving system, an enzyme complex in a pathway of glycine. Mutations in the DLD gene can lead to dldH and deficiency of these for Maple Syrup Urine Disease Type III and Leigh syndrome.

DldH and dihydrolipoamide are together able to denitrosieren S- nitroso groups in proteins, a task that is usually provided by thioredoxin. DldH may also reduce nitro groups to DNA and proteins to the amine and is part of the metabolism of reactive nitrogen species.

Human Genome Organisation Leigh's disease FEBS Letters Digital Object Identifier
240001
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