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].
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Name | OMIM ID | Ensembl ID | HGNC ID | PHARMGKB ID | Map Location |
---|---|---|---|---|---|
OMIM IDMIM:613022 Ensembl IDEnsembl:ENSG00000105953 HGNC IDHGNC:HGNC:8124 PHARMGKB IDPA31910 Map Location7p13 |
GO terms in OGDH
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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 |
Gene expression in normal tissue: OGDH
Gene-model tissue-cancer distribution: Bubble Plot
Gene-drug pathway distribution
Pathways in OGDH
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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 |
Gene-Drug: Aster Plot
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Drug ID | Drug Name | Model Num. |
---|---|---|
iGMDRD353 | PD0325901 | 1 |
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Gene in drug-gene network: Network Plot

Gene-drug targets distribution
Gene Structure: PDB
Models in OGDH
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Model | Level | Reference ID | Tissue | Cancer | Drug | Clinical Response | Source | |
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No matching records found |