Remarkably, cancer-associated mutations in some TCA cycle enzymes, including fumarate hydratase (FH, linked to hereditary renal cell carcinomas, RCC), succinate dehydrogenase (SDH, linked to paraganglioma) and isocitrate dehydrogenases (IDH, linked to glioma and acute myeloid leukemia, AML) dictate specific metabolic patterns by altering the accumulation of these signaling intermediates (Yong et al., 2020). Here, IDH3A is linked to paraganglioma.