Indeed, mutations of the genes encoding the isocitrate dehydrogenase enzymes (IDH1 or IDH2), succinate dehydrogenase and fumarate hydratase, leading to accumulation of the 2-hydroxyglutarate, succinate and fumarate oncometabolites, have been reported in several types of cancer, resulting in a state of pseudohypoxia due to the inhibition of prolyl-hydroxylase enzymes [6, 17–20]. The gene discussed is IDH2; the disease is cancer.