R-2-hydroxyglutarate as the key effector of IDH mutations promoting oncogenesis

The tumor-associated isocitrate dehydrogenase (IDH) mutants are unique in that they have lost their normal catalytic activity and gained a novel function to produce R-2-hydroxyglutarate (R-2-HG). A recent study now shows that R-2-HG can reversibly promote leukemogenesis in vitro, suggesting a therapeutic potential of targeting mutant IDH1 and IDH2.