Succinate Dehydrogenase Loss in Familial Paraganglioma: Biochemistry, Genetics, and Epigenetics

Figure 5

Structural similarity between TCA cycle oncometabolites. IDH gain of function mutations produces 2-hydroxy-α-KG. SDH and FH mutations lead to accumulation of succinate and fumarate, respectively. MDH2 mutations result in increase of malate and fumarate.