DLD Lipoamide dehydrogenase is a component of the glycine cleavage system as well as an E3 component of three alpha-ketoacid dehydrogenase complexes (pyruvate-, alpha-ketoglutarate-, and branched-chain amino acid-dehydrogenase complex). The 2-oxoglutarate dehydrogenase complex is mainly active in the mitochondrion. A fraction of the 2-oxoglutarate dehydrogenase complex also localizes in the nucleus and is required for lysine succinylation of histones: associates with KAT2A on chromatin and provides succinyl-CoA to histone succinyltransferase KAT2A. In monomeric form may have additional moonlighting function as serine protease. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction. Belongs to the class-I pyridine nucleotide-disulfide oxidoreductase family. 3 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - glycine, serine and threonine; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Carbohydrate Metabolism - pyruvate; EC; Mitochondrial; Oxidoreductase
Chromosomal Location of human Ortholog: 7q31.1
Cellular Component:  acetyltransferase complex; acrosomal matrix; mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrial pyruvate dehydrogenase complex; mitochondrion; motile cilium; nucleus; oxoglutarate dehydrogenase complex; pyruvate dehydrogenase complex
Molecular Function:  dihydrolipoyl dehydrogenase activity; flavin adenine dinucleotide binding; protein binding; pyruvate dehydrogenase (NAD+) activity
Biological Process:  acetyl-CoA biosynthetic process from pyruvate; branched-chain amino acid catabolic process; gastrulation; histone succinylation; mitochondrial electron transport, NADH to ubiquinone; proteolysis; regulation of membrane potential; sperm capacitation
Disease: Dihydrolipoamide Dehydrogenase Deficiency
Reference #:  P09622 (UniProtKB)
Alt. Names/Synonyms: diaphorase; Dihydrolipoamide dehydrogenase; Dihydrolipoyl dehydrogenase, mitochondrial; DLD; DLDD; DLDH; E3; E3 component of pyruvate dehydrogenase complex, 2-oxo-glutarate complex, branched chain keto acid dehydrogenase complex; epididymis secretory sperm binding protein; GCSL; Glycine cleavage system L protein; glycine cleavage system protein L; LAD; lipoamide dehydrogenase; lipoamide reductase; lipoyl dehydrogenase; OGDC-E3; PCD-E3; PHE3
Gene Symbols: DLD
Molecular weight: 54,177 Da
Basal Isoelectric point: 7.95  Predict pI for various phosphorylation states
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Protein Structure Not Found.

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