Gene Symbol: Ogdh
Description: oxoglutarate dehydrogenase
Alias: 2-oxoglutarate dehydrogenase, mitochondrial, 2-oxoglutarate dehydrogenase E1 component, mitochondrial, 2-oxoglutarate dehydrogenase complex component E1, OGDC-E1, alpha-ketoglutarate dehydrogenase, oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide), oxoglutarate dehydrogenase (lipoamide)
Species: rat
Products:     Ogdh

Top Publications

  1. Starkov A, Fiskum G, Chinopoulos C, Lorenzo B, Browne S, Patel M, et al. Mitochondrial alpha-ketoglutarate dehydrogenase complex generates reactive oxygen species. J Neurosci. 2004;24:7779-88 pubmed
    ..The data strongly indicate that KGDHC is a primary site of ROS production in normally functioning mitochondria. ..
  2. Sadakata T, Furuichi T. Identification and mRNA expression of Ogdh, QP-C, and two predicted genes in the postnatal mouse brain. Neurosci Lett. 2006;405:217-22 pubmed
    ..Sequence analysis revealed that d1401 and c1502 were derivatives of Ogdh (oxoglutarate dehydrogenase) and QP-C (ubiquinol-cytochrome c reductase complex ubiquinone-binding protein), ..
  3. Tsepkova P, Artiukhov A, Boyko A, Aleshin V, Mkrtchyan G, Zvyagintseva M, et al. Thiamine Induces Long-Term Changes in Amino Acid Profiles and Activities of 2-Oxoglutarate and 2-Oxoadipate Dehydrogenases in Rat Brain. Biochemistry (Mosc). 2017;82:723-736 pubmed publisher
  4. Ono K, Hakozaki M, Kimura A, Kochi H. Purification, resolution, and reconstitution of rat liver branched-chain alpha-keto acid dehydrogenase complex. J Biochem. 1987;101:19-27 pubmed
    ..Apparent Km values were 10-12 microM for the branched-chain alpha-keto acids, 2.2 mM for pyruvate, and 2.5 mM for alpha-ketoglutarate. ..
  5. Bruschi S, Lindsay J, Crabb J. Mitochondrial stress protein recognition of inactivated dehydrogenases during mammalian cell death. Proc Natl Acad Sci U S A. 1998;95:13413-8 pubmed
    ..These observations confirm the role of mammalian stress proteins in the recognition of abnormal proteins and provide supporting evidence for reactive metabolite-induced cell death by modification of critical protein targets. ..
  6. Oktay Y, Dioum E, Matsuzaki S, Ding K, Yan L, Haller R, et al. Hypoxia-inducible factor 2alpha regulates expression of the mitochondrial aconitase chaperone protein frataxin. J Biol Chem. 2007;282:11750-6 pubmed
    ..These findings expand upon the previously defined role of HIF-2alpha in the cellular response to oxidative stress and identify a novel link of HIF-2alpha with mitochondrial homeostasis. ..
  7. Bunik V, Kaehne T, Degtyarev D, Shcherbakova T, Reiser G. Novel isoenzyme of 2-oxoglutarate dehydrogenase is identified in brain, but not in heart. FEBS J. 2008;275:4990-5006 pubmed publisher
    2-Oxoglutarate dehydrogenase (OGDH) is the first and rate-limiting component of the multienzyme OGDH complex (OGDHC) whose malfunction is associated with neurodegeneration...
  8. Denton R, Pullen T, Armstrong C, Heesom K, Rutter G. Calcium-insensitive splice variants of mammalian E1 subunit of 2-oxoglutarate dehydrogenase complex with tissue-specific patterns of expression. Biochem J. 2016;473:1165-78 pubmed publisher
    The 2-oxoglutarate dehydrogenase (OGDH) complex is an important control point in vertebrate mitochondrial oxidative metabolism, including in the citrate cycle and catabolism of alternative fuels including glutamine...
  9. Armstrong C, Anderson J, Denton R. Studies on the regulation of the human E1 subunit of the 2-oxoglutarate dehydrogenase complex, including the identification of a novel calcium-binding site. Biochem J. 2014;459:369-81 pubmed publisher
    ..We conclude that site 1, but not site 2, forms part of a regulatory Ca2+-binding site, which is distinct from other previously described Ca2+-binding sites. ..

More Information


  1. Andersson U, Leighton B, Young M, Blomstrand E, Newsholme E. Inactivation of aconitase and oxoglutarate dehydrogenase in skeletal muscle in vitro by superoxide anions and/or nitric oxide. Biochem Biophys Res Commun. 1998;249:512-6 pubmed
    ..It is suggested that these effects may be responsible for some alterations in skeletal muscle metabolism, and these possibilities are discussed. ..
  2. Sheu K, Blass J. The alpha-ketoglutarate dehydrogenase complex. Ann N Y Acad Sci. 1999;893:61-78 pubmed
    ..We hypothesize that variations in KGDHC that are not deleterious during reproductive life become deleterious with aging, perhaps by predisposing this mitochondrial metabolon to oxidative damage. ..
  3. Bunik V, Kabysheva M, Klimuk E, Storozhevykh T, Pinelis V. Phosphono analogues of 2-oxoglutarate protect cerebellar granule neurons upon glutamate excitotoxicity. Ann N Y Acad Sci. 2009;1171:521-9 pubmed publisher
    ..We investigated the role in these phenomena of 2-oxoglutarate dehydrogenase (OGDH), the enzyme participating in mitochondrial degradation of glutamate...
  4. Quinlan C, Goncalves R, Hey Mogensen M, Yadava N, Bunik V, Brand M. The 2-oxoacid dehydrogenase complexes in mitochondria can produce superoxide/hydrogen peroxide at much higher rates than complex I. J Biol Chem. 2014;289:8312-25 pubmed publisher
    ..However, the 2-oxoglutarate dehydrogenase (OGDH), branched-chain 2-oxoacid dehydrogenase (BCKDH), and pyruvate dehydrogenase (PDH) complexes are also capable of ..
  5. Wang Y, Guo Y, Liu K, Yin Z, Liu R, Xia Y, et al. KAT2A coupled with the ?-KGDH complex acts as a histone H3 succinyltransferase. Nature. 2017;552:273-277 pubmed publisher