Gene Symbol: Idh2
Description: isocitrate dehydrogenase 2 (NADP+), mitochondrial
Alias: E430004F23, IDPm, Idh-2, isocitrate dehydrogenase [NADP], mitochondrial, ICD-M, IDH, IDP, NADP(+)-specific ICDH, NADP+-specific isocitrate dehydrogenase, oxalosuccinate decarboxylase
Species: mouse
Products:     Idh2

Top Publications

  1. Krog H, Wysocka M, Kisielow P, Radzikowski C. Genetic characterization of important mouse strains used in cancer research. Hereditas. 1977;87:201-4 pubmed
  2. Karlstaedt A, Zhang X, Vitrac H, Harmancey R, Vasquez H, Wang J, et al. Oncometabolite d-2-hydroxyglutarate impairs ?-ketoglutarate dehydrogenase and contractile function in rodent heart. Proc Natl Acad Sci U S A. 2016;113:10436-41 pubmed publisher ex vivo studies, we found that increased amounts of the oncometabolite d-2-hydroxyglutarate (D2-HG), produced by IDH2 mutant leukemic cells, cause contractile dysfunction in the heart...
  3. Yang L, Luo H, Vinay P, Wu J. Molecular cloning of the cDNA of mouse mitochondrial NADP-dependent isocitrate dehydrogenase and the expression of the gene during lymphocyte activation. J Cell Biochem. 1996;60:400-10 pubmed documents the molecular cloning of the mouse mitochondrial NADP-dependent isocitrate dehydrogenase (mNADP-IDH) cDNA...
  4. Jo S, Son M, Koh H, Lee S, Song I, Kim Y, et al. Control of mitochondrial redox balance and cellular defense against oxidative damage by mitochondrial NADP+-dependent isocitrate dehydrogenase. J Biol Chem. 2001;276:16168-76 pubmed
    ..We investigated the role of mitochondrial NADP(+)-dependent isocitrate dehydrogenase (IDPm) in controlling the mitochondrial redox balance and subsequent cellular defense against oxidative damage...
  5. Wang F, Travins J, Lin Z, Si Y, Chen Y, Powe J, et al. A small molecule inhibitor of mutant IDH2 rescues cardiomyopathy in a D-2-hydroxyglutaric aciduria type II mouse model. J Inherit Metab Dis. 2016;39:807-820 pubmed
    ..II is a rare neurometabolic disorder caused by germline gain-of-function mutations in isocitrate dehydrogenase 2 (IDH2), resulting in accumulation of D-2-hydroxyglutarate (D2HG)...
  6. Lee S, Kim S, Park K, Lee J, Park J. Increased obesity resistance and insulin sensitivity in mice lacking the isocitrate dehydrogenase 2 gene. Free Radic Biol Med. 2016;99:179-188 pubmed publisher
    ..We recently reported an important role for mitochondrial NADP+-dependent isocitrate dehydrogenase (IDH2) in cellular redox regulation...
  7. Shin A, Kil I, Yang E, Huh T, Yang C, Park J. Regulation of high glucose-induced apoptosis by mitochondrial NADP+-dependent isocitrate dehydrogenase. Biochem Biophys Res Commun. 2004;325:32-8 pubmed
    ..oxidative damage is one of the primary functions of mitochondrial NADP(+)-dependent isocitrate dehydrogenase (IDPm) to supply NADPH for antioxidant systems...
  8. Park J, Nagar H, Choi S, Jung S, Kim H, Kang S, et al. IDH2 deficiency impairs mitochondrial function in endothelial cells and endothelium-dependent vasomotor function. Free Radic Biol Med. 2016;94:36-46 pubmed publisher
    Mitochondrial NADP(+)-dependent isocitrate dehydrogenase (IDH2) plays an essential role protecting cells against oxidative stress-induced damage...
  9. Ogawara Y, Katsumoto T, Aikawa Y, Shima Y, Kagiyama Y, Soga T, et al. IDH2 and NPM1 Mutations Cooperate to Activate Hoxa9/Meis1 and Hypoxia Pathways in Acute Myeloid Leukemia. Cancer Res. 2015;75:2005-16 pubmed publisher
    IDH1 and IDH2 mutations occur frequently in acute myeloid leukemia (AML) and other cancers...

More Information


  1. Ku H, Ahn Y, Lee J, Park K, Park J. IDH2 deficiency promotes mitochondrial dysfunction and cardiac hypertrophy in mice. Free Radic Biol Med. 2015;80:84-92 pubmed publisher
    ..we have demonstrated that one of the primary functions of mitochondrial NADP(+)-dependent isocitrate dehydrogenase (IDH2) is to control the mitochondrial redox balance, and thereby mediate the cellular defense against oxidative damage, ..
  2. Bogdanovic E, Sadri A, Catapano M, Vance J, Jeschke M. IDH1 regulates phospholipid metabolism in developing astrocytes. Neurosci Lett. 2014;582:87-92 pubmed publisher
    ..In conclusion, our results reveal a role for IDH1 in the synthesis/turnover of phospholipids in developing astrocytes and highlight the lipid alterations resulting from the loss of wild-type IDH1 activity. ..
  3. Kats L, Reschke M, Taulli R, Pozdnyakova O, Burgess K, Bhargava P, et al. Proto-oncogenic role of mutant IDH2 in leukemia initiation and maintenance. Cell Stem Cell. 2014;14:329-41 pubmed publisher
    Mutations in the metabolic enzymes isocitrate dehydrogenase-1 (IDH1) and IDH2 that produce the oncometabolite D-2-hydroxyglutarate (2-HG) occur frequently in human acute myeloid leukemia (AML)...
  4. Lu Q, Minard K, McAlister Henn L. Dual compartmental localization and function of mammalian NADP+-specific isocitrate dehydrogenase in yeast. Arch Biochem Biophys. 2008;472:17-25 pubmed publisher
    Isozymes of NADP+-specific isocitrate dehydrogenase (IDP) provide NADPH in cytosolic, mitochondrial, and peroxisomal compartments of eukaryotic cells...
  5. Stallings R, Siciliano M. Confirmational, provisional, and/or regional assignment of 15 enzyme loci onto Chinese hamster autosomes 1, 2, and 7. Somatic Cell Genet. 1981;7:683-98 pubmed
    ..The assignments provide markers for the study of the genetic consequences of chromosomal rearrangements in Chinese hamster cell lines and support the concept of conservation of mammalian autosomal linkage groups. ..
  6. Lemonnier F, Cairns R, Inoue S, Li W, Dupuy A, Broutin S, et al. The IDH2 R172K mutation associated with angioimmunoblastic T-cell lymphoma produces 2HG in T cells and impacts lymphoid development. Proc Natl Acad Sci U S A. 2016;113:15084-15089 pubmed publisher
    Oncogenic isocitrate dehydrogenase (IDH)1 and IDH2 mutations at three hotspot arginine residues cause an enzymatic gain of function that leads to the production and accumulation of the metabolite 2-hydroxyglutarate (2HG), which ..
  7. Lee S, Cha H, Lee S, Kim H, Ku H, Kim S, et al. Idh2 deficiency accelerates renal dysfunction in aged mice. Biochem Biophys Res Commun. 2017;493:34-39 pubmed publisher
    ..for generating mitochondrial NADPH is mitochondrial NADP+-dependent isocitrate dehydrogenase (IDH2)...
  8. Lalley P, Minna J, Francke U. Conservation of autosomal gene synteny groups in mouse and man. Nature. 1978;274:160-3 pubmed
  9. Crespo I, Vital A, Gonzalez Tablas M, Patino M, Otero A, Lopes M, et al. Molecular and Genomic Alterations in Glioblastoma Multiforme. Am J Pathol. 2015;185:1820-33 pubmed publisher
    ..Herein, we review the most relevant genetic alterations of primary versus secondary GBM, the specific signaling pathways involved, and the overall genomic profile of this genetically heterogeneous group of malignant tumors. ..
  10. Yu W, Dittenhafer Reed K, Denu J. SIRT3 protein deacetylates isocitrate dehydrogenase 2 (IDH2) and regulates mitochondrial redox status. J Biol Chem. 2012;287:14078-86 pubmed publisher
    ..The mitochondrial matrix protein isocitrate dehydrogenase 2 (IDH2) is a major source of NADPH...
  11. Fu X, Huang X, Li P, Chen W, Xia M. 7-Ketocholesterol inhibits isocitrate dehydrogenase 2 expression and impairs endothelial function via microRNA-144. Free Radic Biol Med. 2014;71:1-15 pubmed publisher
    ..this study was to determine the effects and mechanisms of 7-ketocholesterol (7-KC) on isocitrate dehydrogenase 2 (IDH2) and its impact on endothelial function in both human aortic endothelial cells (HAECs) and C57BL/6J mice...
  12. Kim S, Kim S, Ku H, Jeon Y, Lee H, Lee J, et al. Suppression of tumorigenesis in mitochondrial NADP(+)-dependent isocitrate dehydrogenase knock-out mice. Biochim Biophys Acta. 2014;1842:135-43 pubmed publisher
    ..oxidative damage is one of the primary functions of mitochondrial NADP(+)-dependent isocitrate dehydrogenase (IDH2) that supplies NADPH for antioxidant systems...
  13. Shih A, Meydan C, Shank K, Garrett Bakelman F, Ward P, Intlekofer A, et al. Combination Targeted Therapy to Disrupt Aberrant Oncogenic Signaling and Reverse Epigenetic Dysfunction in IDH2- and TET2-Mutant Acute Myeloid Leukemia. Cancer Discov. 2017;7:494-505 pubmed publisher
    ..myeloid leukemias (AML) have identified mutations that drive altered DNA methylation, including TET2 and IDH2 Here, we show that models of AML resulting from TET2 or IDH2 mutations combined with FLT3<..
  14. Kim S, Yoo Y, Lee J, Park J. Mitochondrial NADP(+)-dependent isocitrate dehydrogenase knockdown inhibits tumorigenicity of melanoma cells. Biochem Biophys Res Commun. 2014;451:246-51 pubmed publisher
    ..Mitochondrial NADP(+)-dependent isocitrate dehydrogenase (IDH2), an NADPH-generating enzyme, is one of the major antioxidant and redox regulators in mitochondria...
  15. Kim H, Kim S, Cha H, Kim S, Lee J, Park J. IDH2 deficiency promotes mitochondrial dysfunction and dopaminergic neurotoxicity: implications for Parkinson's disease. Free Radic Res. 2016;50:853-60 pubmed publisher
    ..One of these factors, mitochondrial NADP(+)-dependent isocitrate dehydrogenase (IDH2), has been implicated in the regulation of mitochondrial redox balance and reduction of oxidative stress-induced ..
  16. Chen C, Liu Y, Lu C, Cross J, Morris J, Shroff A, et al. Cancer-associated IDH2 mutants drive an acute myeloid leukemia that is susceptible to Brd4 inhibition. Genes Dev. 2013;27:1974-85 pubmed publisher
    Somatic mutations in the isocitrate dehydrogenase (IDH) genes IDH1 and IDH2 occur frequently in acute myeloid leukemia (AML) and other cancers...
  17. Lee S, Cha H, Kim H, Lee J, Park J. Amelioration of late-onset hepatic steatosis in IDH2-deficient mice. Free Radic Res. 2017;51:368-374 pubmed publisher
    ..Recently, we reported that mitochondrial NADP+-dependent isocitrate dehydrogenase, encoded by the IDH2, plays an important role in the regulation of redox balance and oxidative stress levels, which are tightly ..
  18. Lalley P, Francke U, Minna J. Comparative gene mapping in man and mouse: assignment of the genes for lactate dehydrogenase-A, peptidase-D, and isocitrate dehydrogenase-2 to mouse chromosome 7. Cytogenet Cell Genet. 1978;22:577-80 pubmed