Protein Name dihydrolipoamide dehydrogenase
Organism Homo sapiens
Gene ID 1738
Gene Symbol


UniProt P09622 (DLDH_HUMAN), A0A024R713 (A0A024R713_HUMAN), E9PEX6 (E9PEX6_HUMAN)
Relationships Total Number of functionally related compound(s) : 301
Total Number of Articles : 264

dihydrolipoamide dehydrogenase

Gene Summary

This gene encodes a member of the class-I pyridine nucleotide-disulfide oxidoreductase family. The encoded protein has been identified as a moonlighting protein based on its ability to perform mechanistically distinct functions. In homodimeric form, the encoded protein functions as a dehydrogenase and is found in several multi-enzyme complexes that regulate energy metabolism. However, as a monomer, this protein can function as a protease. Mutations in this gene have been identified in patients with E3-deficient maple syrup urine disease and lipoamide dehydrogenase deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]

  • dihydrolipoyl dehydrogenase, mitochondrial
  • E3 component of pyruvate dehydrogenase complex, 2-oxo-glutarate complex, branched chain keto acid dehydrogenase complex
  • diaphorase
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