IDH2
Summary: Isocitrate dehydrogenases catalyze the oxidative decarboxylation of isocitrate to 2-oxoglutarate. These enzymes belong to two distinct subclasses, one of which utilizes NAD(+) as the electron acceptor and the other NADP(+). Five isocitrate dehydrogenases have been reported: three NAD(+)-dependent isocitrate dehydrogenases, which localize to the mitochondrial matrix, and two NADP(+)-dependent isocitrate dehydrogenases, one of which is mitochondrial and the other predominantly cytosolic. Each NADP(+)-dependent isozyme is a homodimer. The protein encoded by this gene is the NADP(+)-dependent isocitrate dehydrogenase found in the mitochondria. It plays a role in intermediary metabolism and energy production. This protein may tightly associate or interact with the pyruvate dehydrogenase complex. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Feb 2014].
| Name | OMIM ID | Ensembl ID | HGNC ID | PHARMGKB ID | Map Location |
|---|---|---|---|---|---|
| isocitrate dehydrogenase (NADP(+)) 2, mitochondrial | MIM:147650 | Ensembl:ENSG00000182054 | HGNC:HGNC:5383 | PA29631 | 15q26.1 |
Gene Categories:
CLINICALLY ACTIONABLEGO terms in IDH2
| Term Type | Evidence Type | GO Term ID | GO Des. |
|---|---|---|---|
| CC | HDA | GO:0005739 | mitochondrion |
| CC | IDA | GO:0005739 | mitochondrion |
| CC | TAS | GO:0005759 | mitochondrial matrix |
| CC | IEA | GO:0005777 | peroxisome |
| CC | IEA | GO:0005829 | cytosol |
| CC | HDA | GO:0070062 | extracellular exosome |
| BP | NAS | GO:0005975 | carbohydrate metabolic process |
| BP | IEA | GO:0006097 | glyoxylate cycle |
| BP | TAS | GO:0006099 | tricarboxylic acid cycle |
| BP | ISS | GO:0006102 | isocitrate metabolic process |
| BP | ISS | GO:0006103 | 2-oxoglutarate metabolic process |
| BP | IEA | GO:0006741 | NADP biosynthetic process |
| BP | IEA | GO:0060253 | negative regulation of glial cell proliferation |
| BP | IEA | GO:1903976 | negative regulation of glial cell migration |
| BP | IEA | GO:1904465 | negative regulation of matrix metallopeptidase secretion |
| MF | ISS | GO:0000287 | magnesium ion binding |
| MF | EXP | GO:0004450 | isocitrate dehydrogenase (NADP+) activity |
| MF | IDA | GO:0004450 | isocitrate dehydrogenase (NADP+) activity |
| MF | ISS | GO:0004450 | isocitrate dehydrogenase (NADP+) activity |
| MF | IEA | GO:0051287 | NAD binding |
Gene expression in normal tissue: IDH2
Gene-model tissue-cancer distribution: Bubble Plot
Gene-drug pathway distribution
Pathways in IDH2
| 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-1592230 | Mitochondrial biogenesis |
| reactome | R-HSA-1852241 | Organelle biogenesis and maintenance |
| reactome | R-HSA-2151201 | Transcriptional activation of mitochondrial biogenesis |
| reactome | R-HSA-71403 | Citric acid cycle (TCA cycle) |
| reactome | R-HSA-71406 | Pyruvate metabolism and Citric Acid (TCA) cycle |
| kegg | hsa00020 | Citrate cycle (TCA cycle) - Homo sapiens (human) |
| kegg | hsa00480 | Glutathione metabolism - Homo sapiens (human) |
| kegg | hsa04146 | Peroxisome - Homo sapiens (human) |
| wikipathways | WP3961 | fig-met-1-last-solution |
| wikipathways | WP78 | TCA Cycle |
| 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 |
Gene-Drug: Aster Plot
| Drug ID | Drug Name | Model Num. |
|---|---|---|
| iGMDRD85 | Ursolic acid | 3 |
| iGMDRD239 | Dasatinib | 2 |
| iGMDRD560 | MK-2206 | 3 |
| iGMDRD781 | Sirolimus | 2 |
| iGMDRD126 | Tipifarnib | 5 |
| iGMDRD772 | BRD4770 | 3 |
| iGMDRD889 | Compound 7d-cis | 1 |
| iGMDRD923 | IDH2 inhibitor | 2 |
| iGMDRD524 | ISX-9 | 3 |
| iGMDRD677 | ABT-199 (GDC-0199) | 1 |
| iGMDRD782 | DC-45-A2 | 2 |
| iGMDRD790 | AG-221 | 7 |
| iGMDRD294 | Batimastat | 2 |
| iGMDRD226 | ML006 | 3 |
| iGMDRD318 | PAC-1 | 3 |
| iGMDRD255 | SB225002 | 3 |
Gene in drug-gene network: Network Plot
Gene-drug targets distribution
Gene Structure: PDB
Models in IDH2
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