IDH catalyzes the oxidative carboxylation of isocitrate to produce α-KG, whereas its mutations result in the gain of function, converting α-KG to the oncometabolite 2-HG.1297 In 2017, the FDA approved enasidenib, a first-in-class IDH2 mutation inhibitor developed by Celegene, for the treatment of recurrent or refractory AML with IDH2 mutation. Here, IDH2 is linked to acute myeloid leukemia.