dihydrolipoamide dehydrogenase

(redirected from Dihydrolipoyl dehydrogenase)

di·hy·dro·lip·o·am·ide de·hy·dro·gen·ase

(dī-hī'drō-lip-ō-am'id dē-hī'drō-jen-ās'),
A flavoenzyme oxidizing dihydrolipoamide at the expense of NAD+; completes the oxidative decarboxylation of pyruvate; a part of several enzyme complexes (for example, α-ketoglutarate dehydrogenase complex). Decreased activity leads to neuronal loss in brain resulting in psychomotor retardation.

dihydrolipoamide dehydrogenase

The flavoprotein catalyst of the reoxidation of nicotinamide-adenine dinucleotide (NAD) or nicotinamide-adenine dinucleotide phosphate (NADP) by the mitochondrial electron transport chain. Synonym: diaphorase
References in periodicals archive ?
A defect in branched-chain amino acid metabolism in a patient with congenital lactic acidosis due to dihydrolipoyl dehydrogenase deficiency.
Lactic acidemia, neurologic deterioration and carbohydrate dependence in a girl with dihydrolipoyl dehydrogenase deficiency.
The abnormal excretion of these branched compounds implies the need for differentiation from dihydrolipoyl dehydrogenase deficiency.

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