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OGDH  -  oxoglutarate (alpha-ketoglutarate)...

Homo sapiens

Synonyms: 2-oxoglutarate dehydrogenase complex component E1, 2-oxoglutarate dehydrogenase, mitochondrial, AKGDH, Alpha-ketoglutarate dehydrogenase, E1k, ...
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Disease relevance of OGDH


Psychiatry related information on OGDH


High impact information on OGDH


Chemical compound and disease context of OGDH


Biological context of OGDH


Anatomical context of OGDH


Associations of OGDH with chemical compounds

  • 2-Oxoglutarate dehydrogenase (lipoamide) (( OGDH: 2-oxoglutarate:lipoamide 2-oxidoreductase (decarboxylating and acceptor-succinylating), EC )) is a component enzyme of the 2-oxoglutarate dehydrogenase complex [1].
  • The results of primer extension analysis imply that the OGDH transcription start point is a thymine residue 55 bp upstream from the ATG start codon [18].
  • Human 2-oxoglutarate dehydrogenase (OGDH) is an E1-component of the OGDH multi-enzyme complex and catalyzes both the ThDP-dependent decarboxylation of 2-oxoglutarate and the subsequent reductive succinylation of the lipoyl moiety which is covalently bound to the E2 component, dihydrolipoamide succinyltransferase [23].
  • In the present study, the effects of 4-hydroxy-2-nonenal (HNE) on highly purified pyruvate dehydrogenase complex (PDC) and its catalytic components in vitro and on PDC, alpha-ketoglutarate dehydrogenase complex (KGDC), and the branched-chain alpha-keto acid dehydrogenase complex (BCKDC) activities in cultured human HepG2 cells were investigated [24].
  • One of these peptides, QLSPFPFDL (QL9), elicits more vigorous cytolytic responses than two previously identified naturally processed peptides with overlapping sequences, LSPFPFDL (p2Ca) and VAITRIEQLSPFPFDL (p2Cb), from OGDH [20].

Physical interactions of OGDH


Enzymatic interactions of OGDH

  • Analysis of the pattern of release of E1 and E1' polypeptides from the OGDC during tryptic inactivation suggests that both polypeptide chains of individual E1 homodimers must be cleaved to permit the dissociation of the E1 and E3 components [25].

Regulatory relationships of OGDH


Other interactions of OGDH

  • No significant difference was noted in the activities of oxidative enzymes [malate dehydrogenase (MDH), oxoglutarate dehydrogenase (OGDH), and 3-hydroxyacyl-CoA dehydrogenase (HADH)] [29].
  • KGDHC consists of multiple copies of E1k, E2k, and E3 [3].
  • The dihydrolipoamide succinyltransferase (DLST) gene of the alpha-ketoglutarate dehydrogenase complex (alpha-KGDC) was isolated from a rat genomic DNA library and sequenced [30].
  • Significant biochemical changes occurred in saline-treated stimulated rats compared with shams: with reductions in glutathione, alpha-ketoglutarate dehydrogenase, aconitase, citrate synthase, and complex I activities [31].
  • Exclusion of OGDH and BMP4 as candidate genes in two siblings with autosomal recessive DOOR syndrome [32].

Analytical, diagnostic and therapeutic context of OGDH


  1. Cloning and nucleotide sequence of the cDNA encoding human 2-oxoglutarate dehydrogenase (lipoamide). Koike, K., Urata, Y., Goto, S. Proc. Natl. Acad. Sci. U.S.A. (1992) [Pubmed]
  2. Brain protein and alpha-ketoglutarate dehydrogenase complex activity in Alzheimer's disease. Mastrogiacoma, F., Lindsay, J.G., Bettendorff, L., Rice, J., Kish, S.J. Ann. Neurol. (1996) [Pubmed]
  3. Reduction in the E2k subunit of the alpha-ketoglutarate dehydrogenase complex has effects independent of complex activity. Shi, Q., Chen, H.L., Xu, H., Gibson, G.E. J. Biol. Chem. (2005) [Pubmed]
  4. Immunoreactive levels of alpha-ketoglutarate dehydrogenase subunits in Friedreich's ataxia and spinocerebellar ataxia type 1. Mastrogiacomo, F., LaMarche, J., Dozić, S., Lindsay, G., Bettendorff, L., Robitaille, Y., Schut, L., Kish, S.J. Neurodegeneration : a journal for neurodegenerative disorders, neuroprotection, and neuroregeneration. (1996) [Pubmed]
  5. A challenge for 21st century molecular biology and biochemistry: what are the causes of obligate autotrophy and methanotrophy? Wood, A.P., Aurikko, J.P., Kelly, D.P. FEMS Microbiol. Rev. (2004) [Pubmed]
  6. Thiamine-dependent enzyme changes in the brains of alcoholics: relationship to the Wernicke-Korsakoff syndrome. Butterworth, R.F., Kril, J.J., Harper, C.G. Alcohol. Clin. Exp. Res. (1993) [Pubmed]
  7. Maple syrup urine disease caused by a partial deletion in the inner E2 core domain of the branched chain alpha-keto acid dehydrogenase complex due to aberrant splicing. A single base deletion at a 5'-splice donor site of an intron of the E2 gene disrupts the consensus sequence in this region. Mitsubuchi, H., Nobukuni, Y., Akaboshi, I., Indo, Y., Endo, F., Matsuda, I. J. Clin. Invest. (1991) [Pubmed]
  8. Antibodies against mitochondrial dehydrogenase complexes in primary biliary cirrhosis. Yoshida, T., Bonkovsky, H., Ansari, A., Danner, D. Gastroenterology (1990) [Pubmed]
  9. Truncated product of the bifunctional DLST gene involved in biogenesis of the respiratory chain. Kanamori, T., Nishimaki, K., Asoh, S., Ishibashi, Y., Takata, I., Kuwabara, T., Taira, K., Yamaguchi, H., Sugihara, S., Yamazaki, T., Ihara, Y., Nakano, K., Matuda, S., Ohta, S. EMBO J. (2003) [Pubmed]
  10. Sequences directing dihydrolipoamide dehydrogenase (E3) binding are located on the 2-oxoglutarate dehydrogenase (E1) component of the mammalian 2-oxoglutarate dehydrogenase multienzyme complex. Rice, J.E., Dunbar, B., Lindsay, J.G. EMBO J. (1992) [Pubmed]
  11. Variant tricarboxylic acid cycle in Mycobacterium tuberculosis: identification of alpha-ketoglutarate decarboxylase. Tian, J., Bryk, R., Itoh, M., Suematsu, M., Nathan, C. Proc. Natl. Acad. Sci. U.S.A. (2005) [Pubmed]
  12. Inhibition of alpha-ketoglutarate dehydrogenase activity by a distinct population of autoantibodies recognizing dihydrolipoamide succinyltransferase in primary biliary cirrhosis. Fregeau, D.R., Prindiville, T., Coppel, R.L., Kaplan, M., Dickson, E.R., Gershwin, M.E. Hepatology (1990) [Pubmed]
  13. Deficiency of dihydrolipoyl dehydrogenase (a component of the pyruvate and alpha-ketoglutarate dehydrogenase complexes): a cause of congenital chronic lactic acidosis in infancy. Robinson, B.H., Taylor, J., Sherwood, W.G. Pediatr. Res. (1977) [Pubmed]
  14. Skeletal muscle lactate accumulation and creatine phosphate depletion during heavy exercise in congestive heart failure. Cause of limited exercise capacity? Näveri, H.K., Leinonen, H., Kiilavuori, K., Härkönen, M. Eur. Heart J. (1997) [Pubmed]
  15. Brain alpha-ketoglutarate dehydrogenase complex activity in Alzheimer's disease. Mastrogiacomo, F., Bergeron, C., Kish, S.J. J. Neurochem. (1993) [Pubmed]
  16. Establishment and structural analysis of human mAb to the E2 component of the 2-oxoglutarate dehydrogenase complex generated from a patient with primary biliary cirrhosis. Fukushima, N., Nakamura, M., Matsui, M., Ikematsu, H., Koike, K., Ishibashi, H., Hayashida, K., Niho, Y. Int. Immunol. (1995) [Pubmed]
  17. Localization of the gene (OGDH) coding for the E1k component of the alpha-ketoglutarate dehydrogenase complex to chromosome 7p13-p11.2. Szabo, P., Cai, X., Ali, G., Blass, J.P. Genomics (1994) [Pubmed]
  18. The gene encoding human 2-oxoglutarate dehydrogenase: structural organization and mapping to chromosome 7p13-p14. Koike, K. Gene (1995) [Pubmed]
  19. Relationships between skeletal muscle characteristics and aerobic performance in sedentary and active subjects. Lortie, G., Simoneau, J.A., Hamel, P., Boulay, M.R., Bouchard, C. European journal of applied physiology and occupational physiology. (1985) [Pubmed]
  20. Potent cytolytic response by a CD8+ CTL clone to multiple peptides from the same protein in association with an allogeneic class I MHC molecule. Kageyama, S., Tsomides, T.J., Fukusen, N., Papayannopoulos, I.A., Eisen, H.N., Sykulev, Y. J. Immunol. (2001) [Pubmed]
  21. Abnormality of the alpha-ketoglutarate dehydrogenase complex in fibroblasts from familial Alzheimer's disease. Sheu, K.F., Cooper, A.J., Koike, K., Koike, M., Lindsay, J.G., Blass, J.P. Ann. Neurol. (1994) [Pubmed]
  22. Evidence for a higher glycolytic than oxidative metabolic activity in white matter of rat brain. Morland, C., Henjum, S., Iversen, E.G., Skrede, K.K., Hassel, B. Neurochem. Int. (2007) [Pubmed]
  23. Cloning, structure, chromosomal localization and promoter analysis of human 2-oxoglutarate dehydrogenase gene. Koike, K. Biochim. Biophys. Acta (1998) [Pubmed]
  24. Protection by thiols of the mitochondrial complexes from 4-hydroxy-2-nonenal. Korotchkina, L.G., Yang, H., Tirosh, O., Packer, L., Patel, M.S. Free Radic. Biol. Med. (2001) [Pubmed]
  25. Subunit interactions in the mammalian alpha-ketoglutarate dehydrogenase complex. Evidence for direct association of the alpha-ketoglutarate dehydrogenase and dihydrolipoamide dehydrogenase components. McCartney, R.G., Rice, J.E., Sanderson, S.J., Bunik, V., Lindsay, H., Lindsay, J.G. J. Biol. Chem. (1998) [Pubmed]
  26. Frontal lobe dysfunction in progressive supranuclear palsy: evidence for oxidative stress and mitochondrial impairment. Albers, D.S., Augood, S.J., Park, L.C., Browne, S.E., Martin, D.M., Adamson, J., Hutton, M., Standaert, D.G., Vonsattel, J.P., Gibson, G.E., Beal, M.F. J. Neurochem. (2000) [Pubmed]
  27. Normal glutamate metabolism in Alzheimer's disease fibroblasts deficient in alpha-ketoglutarate dehydrogenase complex activity. Cooper, A.L., Sheu, K.F., Blass, J.P. Dev. Neurosci. (1996) [Pubmed]
  28. The nitration product 5-nitro-gamma-tocopherol is increased in the Alzheimer brain. Williamson, K.S., Gabbita, S.P., Mou, S., West, M., Pye, Q.N., Markesbery, W.R., Cooney, R.V., Grammas, P., Reimann-Philipp, U., Floyd, R.A., Hensley, K. Nitric Oxide (2002) [Pubmed]
  29. Skeletal muscle characteristics in sedentary black and Caucasian males. Ama, P.F., Simoneau, J.A., Boulay, M.R., Serresse, O., Thériault, G., Bouchard, C. J. Appl. Physiol. (1986) [Pubmed]
  30. Isolation and sequence analysis of the rat dihydrolipoamide succinyltransferase gene. Nakano, K., Tanabe, M., Nakagawa, S., Ohta, S., Suzuki, S., Shimura, M., Matuda, S. DNA Seq. (2002) [Pubmed]
  31. Levetiracetam: antiepileptic properties and protective effects on mitochondrial dysfunction in experimental status epilepticus. Gibbs, J.E., Walker, M.C., Cock, H.R. Epilepsia (2006) [Pubmed]
  32. Exclusion of OGDH and BMP4 as candidate genes in two siblings with autosomal recessive DOOR syndrome. van Bever, Y., Balemans, W., Duval, E.L., Jespers, A., Eyskens, F., van Hul, W., Courtens, W. Am. J. Med. Genet. A (2007) [Pubmed]
  33. Relationship between plasma lactate parameters and muscle characteristics in female cyclists. Bishop, D., Jenkins, D.G., McEniery, M., Carey, M.F. Medicine and science in sports and exercise. (2000) [Pubmed]
  34. Interaction between NAD-dependent isocitrate dehydrogenase, alpha-ketoglutarate dehydrogenase complex, and NADH:ubiquinone oxidoreductase. Porpaczy, Z., Sumegi, B., Alkonyi, I. J. Biol. Chem. (1987) [Pubmed]
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