Role of succinate dehydrogenase deficiency and oncometabolites in gastrointestinal stromal tumors

Figure 1 Mutations in metabolic enzymes produce oncometabolites. Shown are genetic mutations in tricarboxylic acid (TCA) cycle enzymes (underscored) involved in generating oncometabolites (bold). Isocitrate dehydrogenase (IDH) mutations are neomorphic, producing proteins with the modified function of producing D-2-hydroxyglutarate (D-2HG), while succinate dehydrogenase (SDH) and fumarate hydrase (FH) mutations are loss-of-function mutations that lead to the accumulation of succinate and fumarate, respectively. α-KG: α-ketoglutarate.