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].

NameOMIM IDEnsembl IDHGNC IDPHARMGKB IDMap Location
isocitrate dehydrogenase (NADP(+)) 1, cytosolicMIM:147700Ensembl:ENSG00000138413HGNC:HGNC:5382PA296302q34

Gene Categories:

CLINICALLY ACTIONABLE

GO terms in IDH1


Term TypeEvidence TypeGO Term IDGO Des.
MFIDAGO:0000287magnesium ion binding
MFEXPGO:0004450isocitrate dehydrogenase (NADP+) activity
MFIDAGO:0004450isocitrate dehydrogenase (NADP+) activity
MFIPIGO:0005102signaling receptor binding
MFIPIGO:0042802identical protein binding
MFIPIGO:0042803protein homodimerization activity
MFHDAGO:0045296cadherin binding
MFIEAGO:0050661NADP binding
MFIEAGO:0051287NAD binding
MFEXPGO:0051990(R)-2-hydroxyglutarate dehydrogenase activity
CCTASGO:0005576extracellular region
CCIDAGO:0005737cytoplasm
CCIEAGO:0005739mitochondrion
CCIDAGO:0005777peroxisome
CCTASGO:0005782peroxisomal matrix
CCIDAGO:0005829cytosol
CCTASGO:0005829cytosol
CCTASGO:0034774secretory granule lumen
CCHDAGO:0070062extracellular exosome
CCTASGO:1904724tertiary granule lumen
CCTASGO:1904813ficolin-1-rich granule lumen
BPIEAGO:0006097glyoxylate cycle
BPIEAGO:0006099tricarboxylic acid cycle
BPIDAGO:0006102isocitrate metabolic process
BPIDAGO:00061032-oxoglutarate metabolic process
BPTASGO:00061032-oxoglutarate metabolic process
BPTASGO:0006625protein targeting to peroxisome
BPTASGO:0006740NADPH regeneration
BPIEAGO:0006749glutathione metabolic process
BPIEAGO:0006979response to oxidative stress
BPIEAGO:0008585female gonad development
BPTASGO:0043312neutrophil degranulation
BPIEAGO:0048545response to steroid hormone
BPIEAGO:0060696regulation of phospholipid catabolic process
BPIEAGO:0071071regulation of phospholipid biosynthetic process

Gene expression in normal tissue: IDH1

Gene-model tissue-cancer distribution: Bubble Plot

Gene-drug pathway distribution

Pathways in IDH1


DatabasePathway IDPathway Des.
wikipathwaysWP100Glutathione metabolism
wikipathwaysWP2002miR-targeted genes in epithelium - TarBase
wikipathwaysWP2003miR-targeted genes in leukocytes - TarBase
wikipathwaysWP2004miR-targeted genes in lymphocytes - TarBase
wikipathwaysWP2006miR-targeted genes in squamous cell - TarBase
wikipathwaysWP2453TCA Cycle and Deficiency of Pyruvate Dehydrogenase complex (PDHc)
wikipathwaysWP3925Amino Acid metabolism
smpdbSMP00654Warburg Effect
smpdbSMP02291The oncogenic action of 2-hydroxyglutarate
smpdbSMP02292The oncogenic action of Succinate
smpdbSMP02295The oncogenic action of Fumarate
smpdbSMP02358The oncogenic action of L-2-hydroxyglutarate in Hydroxygluaricaciduria
smpdbSMP02359The oncogenic action of D-2-hydroxyglutarate in Hydroxygluaricaciduria
kegghsa00020Citrate cycle (TCA cycle) - Homo sapiens (human)
kegghsa00480Glutathione metabolism - Homo sapiens (human)
kegghsa04146Peroxisome - Homo sapiens (human)
kegghsa05230Central carbon metabolism in cancer - Homo sapiens (human)
reactomeR-HSA-1430728Metabolism
reactomeR-HSA-1643685Disease
reactomeR-HSA-168249Innate Immune System
reactomeR-HSA-168256Immune System
reactomeR-HSA-196854Metabolism of vitamins and cofactors
reactomeR-HSA-2978092Abnormal conversion of 2-oxoglutarate to 2-hydroxyglutarate
reactomeR-HSA-389542NADPH regeneration
reactomeR-HSA-392499Metabolism of proteins
reactomeR-HSA-392499Metabolism of proteins
reactomeR-HSA-5668914Diseases of metabolism
reactomeR-HSA-6798695Neutrophil degranulation
reactomeR-HSA-8978934Metabolism of cofactors
reactomeR-HSA-9033241Peroxisomal protein import
reactomeR-HSA-9033241Peroxisomal protein import

Gene-Drug: Aster Plot


Drug IDDrug NameModel Num.
iGMDRD74Idarubicin6
iGMDRD399Selumetinib4
iGMDRD922IDH1 inhibitor3
iGMDRD190Nilotinib1
iGMDRD369TGX-1152
iGMDRD64Parbendazole6
iGMDRD60Quinoclamine3
iGMDRD356PNU-746541
iGMDRD677ABT-199 (GDC-0199)1
iGMDRD451Serdemetan2
iGMDRD8024-methylfasudil4
iGMDRD284Palbociclib1
iGMDRD171Pemetrexed3
iGMDRD44Temozolomide1
iGMDRD30917AAG1
iGMDRD52Rotenone1
iGMDRD870BRD636102
iGMDRD764AG12011
iGMDRD243BPTES1
iGMDRD794GSK3211
iGMDRD193Fqi15
iGMDRD466Chaetocin3
iGMDRD280CYTOCHALASIN B4
iGMDRD136Nutlin-31
iGMDRD151CHM-12
iGMDRD433Lexibulin3
iGMDRD599Salermide1
iGMDRD1008SR-II-138A3
iGMDRD420Leucascandrolide A2
iGMDRD779PRL-3 Inhibitor I2
iGMDRD239Dasatinib2
iGMDRD367Ro 28-16753
iGMDRD837Bevacizumab3
iGMDRD872BRD71372
iGMDRD329Merck604
iGMDRD246NPC266
iGMDRD322FK 8661
iGMDRD202Curcumin2
iGMDRD121GMX17785
iGMDRD137Indisulam5
iGMDRD832BCL2 inhibitor2
iGMDRD105Triptolide3
iGMDRD267Oligomycin A2
iGMDRD85Ursolic acid2
iGMDRD351GW843682X3
iGMDRD352PD-03259011
iGMDRD536PLX-47201
iGMDRD698Neratinib4
iGMDRD237ABT-7516
iGMDRD177Teniposide2
iGMDRD123Isoevodiamine3
iGMDRD154NSC237661
iGMDRD436GSK429286A1
iGMDRD512nutlin 32
iGMDRD726AGI-51982
iGMDRD560MK-22062
iGMDRD405PIK-752

Gene in drug-gene network: Network Plot

Gene-drug targets distribution

Gene Structure: PDB

Models in IDH1

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