IDH1 and IDH2 Mutations in Gliomas

Neuro-Oncology (LE Abrey, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Neuro-Oncology


Mutations in isocitrate dehydrogenase (IDH) 1 and 2, originally discovered in 2008, occur in the vast majority of low-grade gliomas and secondary high-grade gliomas. These mutations, which occur early in gliomagenesis, change the function of the enzymes, causing them to produce 2-hydroxyglutarate, a possible oncometabolite, and to not produce NADPH. IDH mutations are oncogenic, although whether the mechanism is through alterations in hydroxylases, redox potential, cellular metabolism, or gene expression is not clear. The mutations also drive increased methylation in gliomas. Gliomas with mutated IDH1 and IDH2 have improved prognosis compared with gliomas with wild-type IDH. Mutated IDH can now be detected by immunohistochemistry and magnetic resonance spectroscopy. No drugs currently target mutated IDH, although this remains an area of active research.


Isocitrate dehydrogenase Astrocytoma Oligodendroglioma Glioblastoma 2-Hydroxyglutarate Carcinogenesis Prognosis Mutations 

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Adam L. Cohen
    • 1
  • Sheri L. Holmen
    • 2
  • Howard Colman
    • 3
  1. 1.Division of Medical Oncology, Department of Internal Medicine, Huntsman Cancer InstituteUniversity of UtahSalt Lake CityUSA
  2. 2.Department of Surgery, Huntsman Cancer InstituteUniversity of UtahSalt Lake CityUSA
  3. 3.Department of Neurosurgery, Huntsman Cancer InstituteUniverity of UtahSalt Lake CityUSA

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