More recently, AML associated heterozygous somatic mutations of isocitrate dehydrogenase 1 or 2 (IDH1 or 2) were shown to result in a gain of function enzyme that uses alpha-ketoglutarate (αKG) as a substrate to generate the oncometabolite 2-hydroxyglutarate (2HG) [5]. The gene discussed is IDH1; the disease is acute myeloid leukemia.