Figure 2.

Alterations in enzymatic activities leading to the accumulation of oncometabolites. Gain-of-function mutations in IDH1/2 (mIDH1/2) lead to the production of D-2-hydroxyglutarate. mIDH1/2 can be pharmacologically inhibited. Loss-of-function mutations in SDH and FH lead to the accumulation of succinate and fumarate, respectively. CS: citrate synthase; ACO: aconitase; IDH: isocitrate dehydrogenase; α-KGDH: α-ketoglutarate dehydrogenase; SCS: succinyl-CoA synthetase; SDH: succinate dehydrogenase; FH: fumarate hydratase; MDH: malate dehydrogenase.