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Aliases for IDH2 Gene

Aliases for IDH2 Gene

  • Isocitrate Dehydrogenase 2 (NADP+), Mitochondrial 2 3
  • Oxalosuccinate Decarboxylase 3 4
  • NADP(+)-Specific ICDH 3 4
  • EC 4 63
  • D2HGA2 3 6
  • ICD-M 3 4
  • IDPM 3 6
  • IDH 3 4
  • IDP 3 4
  • Isocitrate Dehydrogenase [NADP], Mitochondrial 3
  • IDHM 3

External Ids for IDH2 Gene

Previous GeneCards Identifiers for IDH2 Gene

  • GC15M087031
  • GC15M084133
  • GC15M088213
  • GC15M088356
  • GC15M088428
  • GC15M090626
  • GC15M066740

Summaries for IDH2 Gene

Entrez Gene Summary for IDH2 Gene

  • 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]

GeneCards Summary for IDH2 Gene

IDH2 (Isocitrate Dehydrogenase 2 (NADP+), Mitochondrial) is a Protein Coding gene. Diseases associated with IDH2 include d-2-hydroxyglutaric aciduria 2 and protoplasmic astrocytoma. Among its related pathways are Metabolism and Carbon metabolism. GO annotations related to this gene include magnesium ion binding and isocitrate dehydrogenase (NADP+) activity. An important paralog of this gene is IDH1.

UniProtKB/Swiss-Prot for IDH2 Gene

  • Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex

Gene Wiki entry for IDH2 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for IDH2 Gene

Genomics for IDH2 Gene

Regulatory Elements for IDH2 Gene

Epigenetics Products

  • DNA Methylation CpG Assay Predesigned for Pyrosequencing in human,mouse,rat

Genomic Location for IDH2 Gene

90,083,045 bp from pter
90,102,504 bp from pter
19,460 bases
Minus strand

Genomic View for IDH2 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for IDH2 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for IDH2 Gene

Proteins for IDH2 Gene

  • Protein details for IDH2 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Isocitrate dehydrogenase [NADP], mitochondrial
    Protein Accession:
    Secondary Accessions:
    • B2R6L6
    • B4DFL2
    • Q96GT3

    Protein attributes for IDH2 Gene

    452 amino acids
    Molecular mass:
    50909 Da
    Name=Mg(2+); Xref=ChEBI:CHEBI:18420; Name=Mn(2+); Xref=ChEBI:CHEBI:29035; Note=Binds 1 Mg(2+) or Mn(2+) ion per subunit.;
    Quaternary structure:
    • Homodimer

    Three dimensional structures from OCA and Proteopedia for IDH2 Gene

    Alternative splice isoforms for IDH2 Gene


neXtProt entry for IDH2 Gene

Proteomics data for IDH2 Gene at MOPED

Post-translational modifications for IDH2 Gene

  • Acetylation at Lys-413 dramatically reduces catalytic activity. Deacetylated by SIRT3.
  • Ubiquitination at Lys166 and Lys272
  • Modification sites at PhosphoSitePlus

Other Protein References for IDH2 Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

Antibody Products

  • Cell Signaling Technology (CST) Antibodies for IDH2 (IDH2)

Domains for IDH2 Gene

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the isocitrate and isopropylmalate dehydrogenases family.
  • Belongs to the isocitrate and isopropylmalate dehydrogenases family.
genes like me logo Genes that share domains with IDH2: view

No data available for Gene Families for IDH2 Gene

Function for IDH2 Gene

Molecular function for IDH2 Gene

GENATLAS Biochemistry:
isocitrate dehydrogenase 2,mitochrondrial matrix,NADPH dependent,46.5 kDa,catalyzing the third step of citric acid cycle
UniProtKB/Swiss-Prot CatalyticActivity:
Isocitrate + NADP(+) = 2-oxoglutarate + CO(2) + NADPH
UniProtKB/Swiss-Prot Function:
Plays a role in intermediary metabolism and energy production. It may tightly associate or interact with the pyruvate dehydrogenase complex

Enzyme Numbers (IUBMB) for IDH2 Gene

Gene Ontology (GO) - Molecular Function for IDH2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000287 magnesium ion binding ISS --
GO:0004450 isocitrate dehydrogenase (NADP+) activity ISS --
GO:0016616 oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor --
GO:0051287 NAD binding IEA --
genes like me logo Genes that share ontologies with IDH2: view

Animal Model Products

miRNA for IDH2 Gene

miRTarBase miRNAs that target IDH2

No data available for Phenotypes , Animal Models , Transcription Factor Targets and HOMER Transcription for IDH2 Gene

Localization for IDH2 Gene

Subcellular locations from UniProtKB/Swiss-Prot for IDH2 Gene


Subcellular locations from

Jensen Localization Image for IDH2 Gene COMPARTMENTS Subcellular localization image for IDH2 gene
Compartment Confidence
mitochondrion 5
chloroplast 2
cytosol 2
peroxisome 2
plasma membrane 2
cytoskeleton 1
endoplasmic reticulum 1
extracellular 1
golgi apparatus 1
lysosome 1
nucleus 1
vacuole 1

Gene Ontology (GO) - Cellular Components for IDH2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA --
GO:0005743 mitochondrial inner membrane IEA --
GO:0005759 mitochondrial matrix TAS --
GO:0070062 extracellular exosome IDA 23533145
genes like me logo Genes that share ontologies with IDH2: view

Pathways for IDH2 Gene

genes like me logo Genes that share pathways with IDH2: view

Pathways by source for IDH2 Gene

PCR Array Products

  • Pathway & Disease-focused RT² Profiler PCR Arrays

Gene Ontology (GO) - Biological Process for IDH2 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005975 carbohydrate metabolic process NAS 8954790
GO:0006097 glyoxylate cycle IEA --
GO:0006099 tricarboxylic acid cycle TAS --
GO:0006102 isocitrate metabolic process ISS --
GO:0006103 2-oxoglutarate metabolic process ISS --
genes like me logo Genes that share ontologies with IDH2: view

Drugs for IDH2 Gene

(1) Drugbank Compounds for IDH2 Gene

Compound Status Synonyms Cas Number PubChem ID Type Actions PubMed IDs
Isocitric Acid
320-77-4 447805 Target experimental

(9) HMDB Compounds for IDH2 Gene

Compound Synonyms Cas Number PubMed IDs
Carbon dioxide
  • Carbon oxide
Hydrogen Ion
  • Hydrogen cation
Isocitric acid
  • 1-Hydroxy-1,2,3-propanetricarboxylate
  • Magnesium
  • Manganese

(2) Novoseek inferred chemical compound relationships for IDH2 Gene

Compound -log(P) Hits PubMed IDs
isocitrate 93.6 20
pyruvate 23.1 2
genes like me logo Genes that share compounds with IDH2: view

Transcripts for IDH2 Gene

Unigene Clusters for IDH2 Gene

Isocitrate dehydrogenase 2 (NADP+), mitochondrial:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for IDH2 Gene

ExUns: 1a · 1b ^ 2 ^ 3 ^ 4a · 4b ^ 5 ^ 6 ^ 7 ^ 8a · 8b ^ 9 ^ 10 ^ 11 ^ 12 ^ 13 ^ 14 ^ 15
SP1: - - - - -
SP2: - - - -
SP3: - - -
SP4: - -
SP5: - -

Relevant External Links for IDH2 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for IDH2 Gene

mRNA expression in normal human tissues for IDH2 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for IDH2 Gene

This gene is overexpressed in Muscle - Skeletal (7.8), Heart - Left Ventricle (7.4), and Liver (4.4).

Protein differential expression in normal tissues for IDH2 Gene

This gene is overexpressed in Heart (10.6).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for IDH2 Gene

SOURCE GeneReport for Unigene cluster for IDH2 Gene Hs.596461

genes like me logo Genes that share expressions with IDH2: view

Expression partners for IDH2 Gene

* - Elite partner

Primer Products

In Situ Assay Products

No data available for mRNA Expression by UniProt/SwissProt for IDH2 Gene

Orthologs for IDH2 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for IDH2 Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia IDH2 35
  • 91.08 (n)
  • 96.24 (a)
IDH2 36
  • 96 (a)
(Canis familiaris)
Mammalia IDH2 35
  • 90.56 (n)
  • 96.37 (a)
IDH2 36
  • 94 (a)
(Mus musculus)
Mammalia Idh2 35
  • 89.97 (n)
  • 95.13 (a)
Idh2 16
Idh2 36
  • 95 (a)
(Pan troglodytes)
Mammalia IDH2 35
  • 99.63 (n)
  • 99.78 (a)
IDH2 36
  • 100 (a)
(Rattus norvegicus)
Mammalia Idh2 35
  • 89.53 (n)
  • 94.69 (a)
(Monodelphis domestica)
Mammalia IDH2 36
  • 88 (a)
(Ornithorhynchus anatinus)
Mammalia IDH2 36
  • 83 (a)
(Gallus gallus)
Aves IDH2 35
  • 83.22 (n)
  • 89.26 (a)
IDH2 36
  • 88 (a)
(Anolis carolinensis)
Reptilia IDH2 36
  • 88 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia idh2 35
  • 75.15 (n)
  • 85.4 (a)
Str.6413 35
(Danio rerio)
Actinopterygii idh2 35
  • 76.91 (n)
  • 84.63 (a)
wufb33c06 35
IDH2 36
  • 61 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP003168 35
  • 68.51 (n)
  • 71.49 (a)
(Caenorhabditis elegans)
Secernentea C34F6.8 37
  • 70 (a)
F59B8.2 37
  • 69 (a)
idh-2 35
  • 62.83 (n)
  • 69.01 (a)
idh-2 36
  • 67 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AAL022W 35
  • 65.5 (n)
  • 65.91 (a)
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0F12342g 35
  • 60.58 (n)
  • 64.25 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes IDP2 35
  • 60.1 (n)
  • 64.84 (a)
IDP1 36
  • 60 (a)
IDP2 36
  • 62 (a)
IDP3 36
  • 60 (a)
IDP3 38
thale cress
(Arabidopsis thaliana)
eudicotyledons ICDH 35
  • 64.04 (n)
  • 68.97 (a)
(Hordeum vulgare)
Liliopsida Hv.4559 35
(Oryza sativa)
Liliopsida Os.2326 35
Os01g0248400 35
  • 64.1 (n)
  • 69.65 (a)
sea squirt
(Ciona savignyi)
Ascidiacea CSA.7852 36
  • 72 (a)
Species with no ortholog for IDH2:
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • fruit fly (Drosophila melanogaster)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for IDH2 Gene

Gene Tree for IDH2 (if available)
Gene Tree for IDH2 (if available)

Paralogs for IDH2 Gene

Paralogs for IDH2 Gene

(1) SIMAP similar genes for IDH2 Gene using alignment to 5 proteins:

genes like me logo Genes that share paralogs with IDH2: view

Variants for IDH2 Gene

Sequence variations from dbSNP and Humsavar for IDH2 Gene

SNP ID Clin Chr 15 pos Sequence Context AA Info Type MAF
rs1802920 -- 90,084,109(-) GTGTT(C/T)CCCCT utr-variant-3-prime
rs2970355 -- 90,085,666(-) TGGGC(C/G)ACAGG intron-variant
rs3934860 -- 90,093,530(+) TACCA(A/G)TGCTG intron-variant
rs4280211 -- 90,103,115(+) AATTG(A/G)TCGGG upstream-variant-2KB
rs4283211 -- 90,103,707(+) AGAGT(A/G)GTCAA upstream-variant-2KB

Structural Variations from Database of Genomic Variants (DGV) for IDH2 Gene

Variant ID Type Subtype PubMed ID
dgv776e1 CNV Complex 17122850
nsv904486 CNV Gain 21882294
dgv112e55 CNV Gain 17911159
dgv777e1 CNV Complex 17122850
nsv442384 CNV CNV 18776908

Relevant External Links for IDH2 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for IDH2 Gene

Disorders for IDH2 Gene

(1) OMIM Diseases for IDH2 Gene (147650)


  • D-2-hydroxyglutaric aciduria 2 (D2HGA2) [MIM:613657]: A neurometabolic disorder causing developmental delay, epilepsy, hypotonia, and dysmorphic features. Both a mild and a severe phenotype exist. The severe phenotype is homogeneous and is characterized by early infantile-onset epileptic encephalopathy and cardiomyopathy. The mild phenotype has a more variable clinical presentation. Diagnosis is based on the presence of an excess of D-2-hydroxyglutaric acid in the urine. {ECO:0000269 PubMed:20847235}. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • Glioma (GLM) [MIM:137800]: Gliomas are benign or malignant central nervous system neoplasms derived from glial cells. They comprise astrocytomas and glioblastoma multiforme that are derived from astrocytes, oligodendrogliomas derived from oligodendrocytes and ependymomas derived from ependymocytes. {ECO:0000269 PubMed:19228619, ECO:0000269 PubMed:25495392}. Note=The gene represented in this entry is involved in disease pathogenesis.

(5) Novoseek inferred disease relationships for IDH2 Gene

Disease -log(P) Hits PubMed IDs
glioblastoma 49.8 5
paraganglioma 48.2 4
glioma 38.4 13
pheochromocytoma 30 4
tumors 6.27 13

Relevant External Links for IDH2

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with IDH2: view

No data available for Genatlas for IDH2 Gene

Publications for IDH2 Gene

  1. IDH1 and IDH2 mutations in gliomas. (PMID: 19228619) Yan H. … Bigner D.D. (N. Engl. J. Med. 2009) 3 4 23
  2. Type and frequency of IDH1 and IDH2 mutations are related to astrocytic and oligodendroglial differentiation and age: a study of 1,010 diffuse gliomas. (PMID: 19554337) Hartmann C. … von Deimling A. (Acta Neuropathol. 2009) 3 23 48
  3. Analysis of IDH1 and IDH2 mutations in Japanese glioma patients. (PMID: 19765000) Sonoda Y. … Tominaga T. (Cancer Sci. 2009) 3 23 48
  4. Rapid and sensitive assessment of the IDH1 and IDH2 mutation status in cerebral gliomas based on DNA pyrosequencing. (PMID: 20131059) Felsberg J. … Reifenberger G. (Acta Neuropathol. 2010) 3 23 48
  5. The common feature of leukemia-associated IDH1 and IDH2 mutations is a neomorphic enzyme activity converting alpha-ketoglutarate to 2-hydroxyglutarate. (PMID: 20171147) Ward P.S. … Thompson C.B. (Cancer Cell 2010) 3 23

Products for IDH2 Gene

Sources for IDH2 Gene

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