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dldh

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Official Full Name
dihydrolipoamide dehydrogenase
Background
Lipoamide dehydrogenase or dihydrolipoamide dehydrogenase (DLD) is a component of the glycine cleavage system as well as of the alpha ketoacid dehydrogenase complexes. It is a homodimer. It belongs to the class I pyridine nucleotide disulfide oxidoreductase family. Defects in DLD are a cause of congenital infantile lactic acidosis. Moreover, defects in DLD are the cause of DLD deficiency; which results in extensive metabolic disturbances, including lactic acidemia, Krebs cycle dysfunction, and impaired branched amino acid degradation. DLD deficiency also causes neurological degeneration due to the sensitivity of the central nervous system to defects in oxidative metabolism.
Synonyms
Lipoamide Dehydrogenase; Diaphorase; Dihydrolipoamide dehydrogenase; Dihydrolipoyl dehydrogenase; DLD; Dihydrolipoyl dehydrogenase mitochondrial; DLDH; E3; E3 component of pyruvate dehydrogenase; E3 component of pyruvate dehydrogenase complex 2 oxo glutarate complex branched chain keto acid dehydrogenase complex; GCSL; Glycine cleavage system L protein; Glycine cleavage system protein L; LAD; Lipoamide reductase; Lipoyl dehydrogenase; PHE 3; PHE3; EC=1.8.1.4; DTD; DTD HUMAN; DT DIAPHORASE; DT DIAPHORASE HUMAN; EC 1.8.1.4 TYPE III; EC 1.8.1.4; EC 1.6.99; EC: 1.6.99; EC 1.6.99.2; IUB: 1.6.99.1; QUINONE REDUCTASE; QUINONE REDUCTASE HUMAN; Diaphorase Clostridium kluyveri; DT Diaphorase from rat; DTD, NQO1, Quinone reductase; dihydrolipoamide dehydrogenase (E3 component of pyruvate dehydrogenase complex, 2 oxo glutarate complex, branched chain keto acid dehydrogenase complex) , GCSL, LAD; dihydrolipoyl dehydrogenase, mitochondrial; E3 component of pyruvate dehydrogenase complex; 2 oxo glutarate complex; branched chain keto acid dehydrogenase complex; E3 component of pyruvate dehydrogenase complex, 2-oxo-glutarate complex, branched chain keto acid dehydrogenase complex; E3, LAD, DLDH, GCSL, PHE3; dihydrolipoamide dehydrogenase (E3 component of pyruvate dehydrogenase complex, 2-oxo-glutarate complex, branched chain keto acid dehydrogenase complex); deltaD; sc:d0402; delta-like protein D; aei; after eight protein