IDH1
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 cytoplasm and peroxisomes. It contains the PTS-1 peroxisomal targeting signal sequence. The presence of this enzyme in peroxisomes suggests roles in the regeneration of NADPH for intraperoxisomal reductions, such as the conversion of 2, 4-dienoyl-CoAs to 3-enoyl-CoAs, as well as in peroxisomal reactions that consume 2-oxoglutarate, namely the alpha-hydroxylation of phytanic acid. The cytoplasmic enzyme serves a significant role in cytoplasmic NADPH production. Alternatively spliced transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Sep 2013].
| Name | OMIM ID | Ensembl ID | HGNC ID | PHARMGKB ID | Map Location |
|---|---|---|---|---|---|
| isocitrate dehydrogenase (NADP(+)) 1, cytosolic | MIM:147700 | Ensembl:ENSG00000138413 | HGNC:HGNC:5382 | PA29630 | 2q34 |
Gene Categories:
CLINICALLY ACTIONABLEGO terms in IDH1
| Term Type | Evidence Type | GO Term ID | GO Des. |
|---|---|---|---|
| MF | IDA | GO:0000287 | magnesium ion binding |
| MF | EXP | GO:0004450 | isocitrate dehydrogenase (NADP+) activity |
| MF | IDA | GO:0004450 | isocitrate dehydrogenase (NADP+) activity |
| MF | IPI | GO:0005102 | signaling receptor binding |
| MF | IPI | GO:0042802 | identical protein binding |
| MF | IPI | GO:0042803 | protein homodimerization activity |
| MF | HDA | GO:0045296 | cadherin binding |
| MF | IEA | GO:0050661 | NADP binding |
| MF | IEA | GO:0051287 | NAD binding |
| MF | EXP | GO:0051990 | (R)-2-hydroxyglutarate dehydrogenase activity |
| CC | TAS | GO:0005576 | extracellular region |
| CC | IDA | GO:0005737 | cytoplasm |
| CC | IEA | GO:0005739 | mitochondrion |
| CC | IDA | GO:0005777 | peroxisome |
| CC | TAS | GO:0005782 | peroxisomal matrix |
| CC | IDA | GO:0005829 | cytosol |
| CC | TAS | GO:0005829 | cytosol |
| CC | TAS | GO:0034774 | secretory granule lumen |
| CC | HDA | GO:0070062 | extracellular exosome |
| CC | TAS | GO:1904724 | tertiary granule lumen |
| CC | TAS | GO:1904813 | ficolin-1-rich granule lumen |
| BP | IEA | GO:0006097 | glyoxylate cycle |
| BP | IEA | GO:0006099 | tricarboxylic acid cycle |
| BP | IDA | GO:0006102 | isocitrate metabolic process |
| BP | IDA | GO:0006103 | 2-oxoglutarate metabolic process |
| BP | TAS | GO:0006103 | 2-oxoglutarate metabolic process |
| BP | TAS | GO:0006625 | protein targeting to peroxisome |
| BP | TAS | GO:0006740 | NADPH regeneration |
| BP | IEA | GO:0006749 | glutathione metabolic process |
| BP | IEA | GO:0006979 | response to oxidative stress |
| BP | IEA | GO:0008585 | female gonad development |
| BP | TAS | GO:0043312 | neutrophil degranulation |
| BP | IEA | GO:0048545 | response to steroid hormone |
| BP | IEA | GO:0060696 | regulation of phospholipid catabolic process |
| BP | IEA | GO:0071071 | regulation of phospholipid biosynthetic process |
Gene expression in normal tissue: IDH1
Gene-model tissue-cancer distribution: Bubble Plot
Gene-drug pathway distribution
Pathways in IDH1
| Database | Pathway ID | Pathway Des. |
|---|---|---|
| wikipathways | WP100 | Glutathione metabolism |
| wikipathways | WP2002 | miR-targeted genes in epithelium - TarBase |
| wikipathways | WP2003 | miR-targeted genes in leukocytes - TarBase |
| wikipathways | WP2004 | miR-targeted genes in lymphocytes - TarBase |
| wikipathways | WP2006 | miR-targeted genes in squamous cell - TarBase |
| wikipathways | WP2453 | TCA Cycle and Deficiency of Pyruvate Dehydrogenase complex (PDHc) |
| wikipathways | WP3925 | Amino Acid metabolism |
| 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 | SMP02358 | The oncogenic action of L-2-hydroxyglutarate in Hydroxygluaricaciduria |
| smpdb | SMP02359 | The oncogenic action of D-2-hydroxyglutarate in Hydroxygluaricaciduria |
| kegg | hsa00020 | Citrate cycle (TCA cycle) - Homo sapiens (human) |
| kegg | hsa00480 | Glutathione metabolism - Homo sapiens (human) |
| kegg | hsa04146 | Peroxisome - Homo sapiens (human) |
| kegg | hsa05230 | Central carbon metabolism in cancer - Homo sapiens (human) |
| reactome | R-HSA-1430728 | Metabolism |
| reactome | R-HSA-1643685 | Disease |
| reactome | R-HSA-168249 | Innate Immune System |
| reactome | R-HSA-168256 | Immune System |
| reactome | R-HSA-196854 | Metabolism of vitamins and cofactors |
| reactome | R-HSA-2978092 | Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarate |
| reactome | R-HSA-389542 | NADPH regeneration |
| reactome | R-HSA-392499 | Metabolism of proteins |
| reactome | R-HSA-392499 | Metabolism of proteins |
| reactome | R-HSA-5668914 | Diseases of metabolism |
| reactome | R-HSA-6798695 | Neutrophil degranulation |
| reactome | R-HSA-8978934 | Metabolism of cofactors |
| reactome | R-HSA-9033241 | Peroxisomal protein import |
| reactome | R-HSA-9033241 | Peroxisomal protein import |
Gene-Drug: Aster Plot
| Drug ID | Drug Name | Model Num. |
|---|---|---|
| iGMDRD74 | Idarubicin | 6 |
| iGMDRD399 | Selumetinib | 4 |
| iGMDRD922 | IDH1 inhibitor | 3 |
| iGMDRD190 | Nilotinib | 1 |
| iGMDRD369 | TGX-115 | 2 |
| iGMDRD64 | Parbendazole | 6 |
| iGMDRD60 | Quinoclamine | 3 |
| iGMDRD356 | PNU-74654 | 1 |
| iGMDRD677 | ABT-199 (GDC-0199) | 1 |
| iGMDRD451 | Serdemetan | 2 |
| iGMDRD802 | 4-methylfasudil | 4 |
| iGMDRD284 | Palbociclib | 1 |
| iGMDRD171 | Pemetrexed | 3 |
| iGMDRD44 | Temozolomide | 1 |
| iGMDRD309 | 17AAG | 1 |
| iGMDRD52 | Rotenone | 1 |
| iGMDRD870 | BRD63610 | 2 |
| iGMDRD764 | AG120 | 11 |
| iGMDRD243 | BPTES | 1 |
| iGMDRD794 | GSK321 | 1 |
| iGMDRD193 | Fqi1 | 5 |
| iGMDRD466 | Chaetocin | 3 |
| iGMDRD280 | CYTOCHALASIN B | 4 |
| iGMDRD136 | Nutlin-3 | 1 |
| iGMDRD151 | CHM-1 | 2 |
| iGMDRD433 | Lexibulin | 3 |
| iGMDRD599 | Salermide | 1 |
| iGMDRD1008 | SR-II-138A | 3 |
| iGMDRD420 | Leucascandrolide A | 2 |
| iGMDRD779 | PRL-3 Inhibitor I | 2 |
| iGMDRD239 | Dasatinib | 2 |
| iGMDRD367 | Ro 28-1675 | 3 |
| iGMDRD837 | Bevacizumab | 3 |
| iGMDRD872 | BRD7137 | 2 |
| iGMDRD329 | Merck60 | 4 |
| iGMDRD246 | NPC26 | 6 |
| iGMDRD322 | FK 866 | 1 |
| iGMDRD202 | Curcumin | 2 |
| iGMDRD121 | GMX1778 | 5 |
| iGMDRD137 | Indisulam | 5 |
| iGMDRD832 | BCL2 inhibitor | 2 |
| iGMDRD105 | Triptolide | 3 |
| iGMDRD267 | Oligomycin A | 2 |
| iGMDRD85 | Ursolic acid | 2 |
| iGMDRD351 | GW843682X | 3 |
| iGMDRD352 | PD-0325901 | 1 |
| iGMDRD536 | PLX-4720 | 1 |
| iGMDRD698 | Neratinib | 4 |
| iGMDRD237 | ABT-751 | 6 |
| iGMDRD177 | Teniposide | 2 |
| iGMDRD123 | Isoevodiamine | 3 |
| iGMDRD154 | NSC23766 | 1 |
| iGMDRD436 | GSK429286A | 1 |
| iGMDRD512 | nutlin 3 | 2 |
| iGMDRD726 | AGI-5198 | 2 |
| iGMDRD560 | MK-2206 | 2 |
| iGMDRD405 | PIK-75 | 2 |
Gene in drug-gene network: Network Plot

Gene-drug targets distribution
Gene Structure: PDB
Models in IDH1

