IDH2, also named as IDP and ICD-M, belongs to the isocitrate and isopropylmalate dehydrogenases family. It plays a role in intermediary metabolism and energy production. IDH2 is a mitochondrial NADP-dependent isocitrate dehydrogenase that catalyzes oxidative decarboxylation of isocitrate to alpha-ketoglutarate, producing NADPH. It may tightly associate or interact with the pyruvate dehydrogenase complex. IDH2 contains an N-terminal mitochondrial addressing sequence and hence is imported to the mitochondrial matrix, although localization to nuclei has also been reported. Moreover, IDH2, like ~20% of other mitochondrial enzymes, is acetylated at lysines, which inactivates the enzymatic activity.