Both enantiomers of 2-HG have been shown to promote malignant progression by their inhibitory action on α-ketoglutarate (αKG)-dependent dioxygenases; they are either synthesized as so-called “oncometabolite” as a result of gain-of-function mutations in IDH1/IDH2 (62, 63) or as pathologic metabolites in Hx cancer cells (64, 65). This evidence concerns the gene IDH2 and cancer.