Human SDH-mutant paraganglioma/pheochromocytoma and FH-deficient renal cancers show SDH/FH-linked hypermethylation and pseudohypoxia signatures, directly tying mitochondrial TCA lesions to epigenetic remodeling in vivo [30,96]. The gene discussed is FH; the disease is hereditary pheochromocytoma-paraganglioma.