Mutation of these genes in cancer leads to a buildup of their substrates; fumarate and succinate in the case of FH and SDH mutations (6, 226), or conversion of their regular substrate to a new “oncometabolite” in the case of mutant IDH1/IDH2 converting isocitrate to 2-hydroxyglutarate (229, 231). This evidence concerns the gene IDH1 and cancer.