Importance Over the past 4 years, our understanding of gliomagenesis and the practice of
neuro-oncology have been radically changed by the discovery of mutations involving the
isocitrate dehydrogenase (IDH) enzymes. IDHmutation has been found to be an inciting
event in gliomagenesis and to have a profound effect on the molecular and genetic route of
oncogenic progression and on clinical outcome. Objectives To review the role of IDH
enzymes in normal physiology and describe aberrations in theIDHpathway that are …