OGDH


Summary: This gene encodes one subunit of the 2-oxoglutarate dehydrogenase complex. This complex catalyzes the overall conversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoA and CO(2) during the Krebs cycle. The protein is located in the mitochondrial matrix and uses thiamine pyrophosphate as a cofactor. A congenital deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Sep 2009].

NameOMIM IDEnsembl IDHGNC IDPHARMGKB IDMap Location
oxoglutarate dehydrogenaseMIM:613022Ensembl:ENSG00000105953HGNC:HGNC:8124PA319107p13

GO terms in OGDH


Term TypeEvidence TypeGO Term IDGO Des.
MFIBAGO:0004591oxoglutarate dehydrogenase (succinyl-transferring) activity
MFIDAGO:0004591oxoglutarate dehydrogenase (succinyl-transferring) activity
MFISSGO:0004591oxoglutarate dehydrogenase (succinyl-transferring) activity
MFIDAGO:0030976thiamine pyrophosphate binding
MFIEAGO:0031072heat shock protein binding
MFIEAGO:0034602oxoglutarate dehydrogenase (NAD+) activity
MFIEAGO:0046872metal ion binding
MFIEAGO:0051087chaperone binding
BPISSGO:0006091generation of precursor metabolites and energy
BPIEAGO:0006096glycolytic process
BPIBAGO:0006099tricarboxylic acid cycle
BPIDAGO:00061032-oxoglutarate metabolic process
BPIDAGO:0006104succinyl-CoA metabolic process
BPIEAGO:0006734NADH metabolic process
BPIEAGO:0021695cerebellar cortex development
BPIEAGO:0021756striatum development
BPIEAGO:0021766hippocampus development
BPIEAGO:0021794thalamus development
BPIEAGO:0021860pyramidal neuron development
BPIEAGO:0022028tangential migration from the subventricular zone to the olfactory bulb
BPIEAGO:0061034olfactory bulb mitral cell layer development
BPIDAGO:0106077histone succinylation
CCIDAGO:0005634nucleus
CCIBAGO:0005739mitochondrion
CCIDAGO:0005739mitochondrion
CCTASGO:0005759mitochondrial matrix
CCIBAGO:0005829cytosol
CCISSGO:0031966mitochondrial membrane
CCIBAGO:0045252oxoglutarate dehydrogenase complex
CCIDAGO:0045252oxoglutarate dehydrogenase complex

Gene expression in normal tissue: OGDH

Gene-model tissue-cancer distribution: Bubble Plot

Gene-drug pathway distribution

Pathways in OGDH


DatabasePathway IDPathway Des.
reactomeR-HSA-1428517The citric acid (TCA) cycle and respiratory electron transport
reactomeR-HSA-1430728Metabolism
reactomeR-HSA-389661Glyoxylate metabolism and glycine degradation
reactomeR-HSA-6788656Histidine, lysine, phenylalanine, tyrosine, proline and tryptophan catabolism
reactomeR-HSA-71064Lysine catabolism
reactomeR-HSA-71291Metabolism of amino acids and derivatives
reactomeR-HSA-71403Citric acid cycle (TCA cycle)
reactomeR-HSA-71406Pyruvate metabolism and Citric Acid (TCA) cycle
wikipathwaysWP2453TCA Cycle and Deficiency of Pyruvate Dehydrogenase complex (PDHc)
wikipathwaysWP3925Amino Acid metabolism
wikipathwaysWP3961fig-met-1-last-solution
wikipathwaysWP465Tryptophan metabolism
wikipathwaysWP78TCA Cycle
smpdbSMP00057Citric Acid Cycle
smpdbSMP00546Congenital lactic acidosis
smpdbSMP00547Fumarase deficiency
smpdbSMP00548Mitochondrial complex II deficiency
smpdbSMP005492-ketoglutarate dehydrogenase complex deficiency
smpdbSMP00550Pyruvate dehydrogenase deficiency (E3)
smpdbSMP00551Pyruvate dehydrogenase deficiency (E2)
smpdbSMP00654Warburg Effect
smpdbSMP02291The oncogenic action of 2-hydroxyglutarate
smpdbSMP02292The oncogenic action of Succinate
smpdbSMP02295The oncogenic action of Fumarate
smpdbSMP02298Glutaminolysis and Cancer
smpdbSMP02358The oncogenic action of L-2-hydroxyglutarate in Hydroxygluaricaciduria
smpdbSMP02359The oncogenic action of D-2-hydroxyglutarate in Hydroxygluaricaciduria
humancycPWY-50842-oxoglutarate decarboxylation to succinyl-CoA
humancycPWY66-398TCA cycle
humancycPWY66-407superpathway of conversion of glucose to acetyl CoA and entry into the TCA cycle
pharmgkbPA165959313Valproic Acid Pathway, Pharmacodynamics
pharmgkbPA165964473Pathway_PA165964473
kegghsa00020Citrate cycle (TCA cycle) - Homo sapiens (human)
kegghsa00310Lysine degradation - Homo sapiens (human)
kegghsa00380Tryptophan metabolism - Homo sapiens (human)

Gene-Drug: Aster Plot


Drug IDDrug NameModel Num.
iGMDRD353PD03259011

Gene in drug-gene network: Network Plot

Gene-drug targets distribution

Gene Structure: PDB

Models in OGDH

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