Figure 2.

Succinate dehydrogenase (SDH) (a) and fumarate hydratase (FH) (b) mutation‐mediated alterations in mitochondrial metabolism. Metabolic pathways that have been shown to be used in cells or tumors containing these mutations are shown. Pathways that are likely to be used, but have not yet been shown are marked with ‘?’ Abbreviations: AcCoA, acetyl CoA; ACO2, mitochondrial aconitase; a‐KG, α‐ketoglutarate; Asp, aspartate; BR, bilirubin; BV, biliverdin; Cit, citrate; Fum, fumarate; Glc, glucose; Gln, glutamine; Glu, glutamate; GSH, reduced glutathione; Gly, glycine; Lac, lactate; OAA, oxaloacetate; PC, pyruvate carboxylase; PDH, pyruvate dehydrogenase; PDK1, pyruvate dehydrogenase kinase 1; Pyr, pyruvate; Suc, succinate.