Interestingly, the mutations in IDH1 and 2 do not impact cancer metabolism per se, as they rather cause the appearance of a neomorphic activity of IDH that perturbs the physiological oxidative decarboxylation of isocitrate to α-KG (see Fig. 4) and promotes the reduction of α-KG to D-2-hydroxyglutarate (D-2HG). Here, IDH2 is linked to cancer.