IDH1 and IDH2 mutations can be found in up to 20% of AML patients and are one of the more frequently occurring mutations in cytogenetically normal AML patients.47 IDH enzymes are key regulators in cellular metabolism and are also important for adaptation to hypoxia, histone demethylation, and DNA modification.191 IDH1 (cytosolic) and IDH2 (mitochondrial) enzymes catalyze the conversion of isocitrate to α-ketoglutarate (α-KG) and produce reduced nicotinamide adenine dinucleotide phosphate (NADPH). The gene discussed is IDH1; the disease is acute myeloid leukemia.