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].
| Name | OMIM ID | Ensembl ID | HGNC ID | PHARMGKB ID | Map Location |
|---|---|---|---|---|---|
| oxoglutarate dehydrogenase | MIM:613022 | Ensembl:ENSG00000105953 | HGNC:HGNC:8124 | PA31910 | 7p13 |
GO terms in OGDH
| Term Type | Evidence Type | GO Term ID | GO Des. |
|---|---|---|---|
| MF | IBA | GO:0004591 | oxoglutarate dehydrogenase (succinyl-transferring) activity |
| MF | IDA | GO:0004591 | oxoglutarate dehydrogenase (succinyl-transferring) activity |
| MF | ISS | GO:0004591 | oxoglutarate dehydrogenase (succinyl-transferring) activity |
| MF | IDA | GO:0030976 | thiamine pyrophosphate binding |
| MF | IEA | GO:0031072 | heat shock protein binding |
| MF | IEA | GO:0034602 | oxoglutarate dehydrogenase (NAD+) activity |
| MF | IEA | GO:0046872 | metal ion binding |
| MF | IEA | GO:0051087 | chaperone binding |
| BP | ISS | GO:0006091 | generation of precursor metabolites and energy |
| BP | IEA | GO:0006096 | glycolytic process |
| BP | IBA | GO:0006099 | tricarboxylic acid cycle |
| BP | IDA | GO:0006103 | 2-oxoglutarate metabolic process |
| BP | IDA | GO:0006104 | succinyl-CoA metabolic process |
| BP | IEA | GO:0006734 | NADH metabolic process |
| BP | IEA | GO:0021695 | cerebellar cortex development |
| BP | IEA | GO:0021756 | striatum development |
| BP | IEA | GO:0021766 | hippocampus development |
| BP | IEA | GO:0021794 | thalamus development |
| BP | IEA | GO:0021860 | pyramidal neuron development |
| BP | IEA | GO:0022028 | tangential migration from the subventricular zone to the olfactory bulb |
| BP | IEA | GO:0061034 | olfactory bulb mitral cell layer development |
| BP | IDA | GO:0106077 | histone succinylation |
| CC | IDA | GO:0005634 | nucleus |
| CC | IBA | GO:0005739 | mitochondrion |
| CC | IDA | GO:0005739 | mitochondrion |
| CC | TAS | GO:0005759 | mitochondrial matrix |
| CC | IBA | GO:0005829 | cytosol |
| CC | ISS | GO:0031966 | mitochondrial membrane |
| CC | IBA | GO:0045252 | oxoglutarate dehydrogenase complex |
| CC | IDA | GO:0045252 | oxoglutarate dehydrogenase complex |
Gene expression in normal tissue: OGDH
Gene-model tissue-cancer distribution: Bubble Plot
Gene-drug pathway distribution
Pathways in OGDH
| Database | Pathway ID | Pathway Des. |
|---|---|---|
| reactome | R-HSA-1428517 | The citric acid (TCA) cycle and respiratory electron transport |
| reactome | R-HSA-1430728 | Metabolism |
| reactome | R-HSA-389661 | Glyoxylate metabolism and glycine degradation |
| reactome | R-HSA-6788656 | Histidine, lysine, phenylalanine, tyrosine, proline and tryptophan catabolism |
| reactome | R-HSA-71064 | Lysine catabolism |
| reactome | R-HSA-71291 | Metabolism of amino acids and derivatives |
| reactome | R-HSA-71403 | Citric acid cycle (TCA cycle) |
| reactome | R-HSA-71406 | Pyruvate metabolism and Citric Acid (TCA) cycle |
| wikipathways | WP2453 | TCA Cycle and Deficiency of Pyruvate Dehydrogenase complex (PDHc) |
| wikipathways | WP3925 | Amino Acid metabolism |
| wikipathways | WP3961 | fig-met-1-last-solution |
| wikipathways | WP465 | Tryptophan metabolism |
| wikipathways | WP78 | TCA Cycle |
| smpdb | SMP00057 | Citric Acid Cycle |
| smpdb | SMP00546 | Congenital lactic acidosis |
| smpdb | SMP00547 | Fumarase deficiency |
| smpdb | SMP00548 | Mitochondrial complex II deficiency |
| smpdb | SMP00549 | 2-ketoglutarate dehydrogenase complex deficiency |
| smpdb | SMP00550 | Pyruvate dehydrogenase deficiency (E3) |
| smpdb | SMP00551 | Pyruvate dehydrogenase deficiency (E2) |
| smpdb | SMP00654 | Warburg Effect |
| smpdb | SMP02291 | The oncogenic action of 2-hydroxyglutarate |
| smpdb | SMP02292 | The oncogenic action of Succinate |
| smpdb | SMP02295 | The oncogenic action of Fumarate |
| smpdb | SMP02298 | Glutaminolysis and Cancer |
| smpdb | SMP02358 | The oncogenic action of L-2-hydroxyglutarate in Hydroxygluaricaciduria |
| smpdb | SMP02359 | The oncogenic action of D-2-hydroxyglutarate in Hydroxygluaricaciduria |
| humancyc | PWY-5084 | 2-oxoglutarate decarboxylation to succinyl-CoA |
| humancyc | PWY66-398 | TCA cycle |
| humancyc | PWY66-407 | superpathway of conversion of glucose to acetyl CoA and entry into the TCA cycle |
| pharmgkb | PA165959313 | Valproic Acid Pathway, Pharmacodynamics |
| pharmgkb | PA165964473 | Pathway_PA165964473 |
| kegg | hsa00020 | Citrate cycle (TCA cycle) - Homo sapiens (human) |
| kegg | hsa00310 | Lysine degradation - Homo sapiens (human) |
| kegg | hsa00380 | Tryptophan metabolism - Homo sapiens (human) |
Gene in drug-gene network: Network Plot
Gene-drug targets distribution
Gene Structure: PDB
Models in OGDH
CLASSFICATION
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