Isocitrate Dehydrogenase 1 (IDH1)

Isocitrate dehydrogenase 1 (IDH1) EC: is one of three isocitrate dehydrogenase isoforms found in humans. Mutated IDH1 catalyzes α-KG to D-2-hydroxyglutarate (D2HG) and has been linked to tumorigenesis in certain types of cancers.

Literature (4)
Gene Data

Isocitrate Dehydrogenase 1 (IDH1) Inhibitors

Cat. No. Product Name / Activity
7087 AGI 5198
Potent and selective inhibitor of mutant isocitrate dehydrogenase 1 (mIDH1)
7761 Ivosidenib
Potent inhibitor of mutant isocitrate dehydrogenase (mIDH1)


Cat. No. Product Name / Activity
6124 (R)-2-Hydroxyglutaric acid disodium salt
Oncometabolite produced by mutant isocitrate dehydrogenase

Related Targets

    Isocitrate dehydrogenase 1 (IDH1) EC: is one of three isocitrate dehydrogenase isoforms found in humans. IDH1 and IDH2 are homodimeric enzymes found in the cytoplasm and mitochondria, respectively. In their wildtype form, they catalyze the conversion of isocitrate to α-ketoglutarate (α-KG), an essential intermediate in the Krebs cycle. IDH1 and IDH2 are structurally and functionally similar, but they differ substantially from IDH3 (an enzyme that plays a role in the Krebs cycle production of NADH).

    In hypoxic cancer cells, or in those with defects in the electron transport chain, HIF-1 mediates signaling that upregulates PDK1 and Myc. This in turn drives IDH1-mediated reductive metabolism of glutamine, a process that is integral to lipogenesis in cancer cells. Genome sequencing projects have identified that mutations in IDH1 are linked to glioblastomas and acute myeloid leukemias (AML), as well as other cancers. Mutant IDH1 converts α-KG to D-2-hydroxyglutarate (D2HG) resulting in high intracellular levels of D2HG, which have been postulated to act as an oncometabolite, driving tumorigenesis. D2HG competitively blocks α-KG binding at a family of enzymes called 2-OG-dependent dioxygenases, which are regulators of important epigenetic events. Mutant IDH enzymes are strongly associated with hypermethylation of CpG islands in AML and glioblastomas. Furthermore, mutations in IDH1 switch the enzyme's function from NADPH production to NADPH catabolism, thus reducing the prevalence of a critical redox mediator.

    External sources of pharmacological information for Isocitrate Dehydrogenase 1 (IDH1) :

      Literature for Isocitrate Dehydrogenase 1 (IDH1)

      Tocris offers the following scientific literature for Isocitrate Dehydrogenase 1 (IDH1) to showcase our products. We invite you to request* your copy today!

      *Please note that Tocris will only send literature to established scientific business / institute addresses.

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      Cancer Metabolism Poster

      Cancer Metabolism Poster

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      Epigenetics in Cancer Poster

      Epigenetics in Cancer Poster

      This poster summarizes the main epigenetic targets in cancer. The dysregulation of epigenetic modifications has been shown to result in oncogenesis and cancer progression. Unlike genetic mutations, epigenetic alterations are considered to be reversible and thus make promising therapeutic targets.

      Isocitrate Dehydrogenase 1 (IDH1) Gene Data

      Gene Species Gene Symbol Gene Accession No. Protein Accession No.
      IDH1 Human IDH1 NM_005896 O75874
      Mouse Idh1 NM_001111320 O88844
      Rat Idh1 NM_031510 P41562