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IDH family members belong to group of enzymes which catalyze metabolic reactions and epigenetic modifications. Mutations in IDH1 and IDH2 generate an oncometabolite product, 2-hydroxyglutarate (2HG), which has been shown to interfere with metabolic and epigenetic mechanisms responsible for cellular differentiation and apoptosis. Small molecule inhibitors of IDH2, such as enasidenib, may be used in MDS treatment.