Mutations in IDH1 and IDH2 have been implicated in various cancers, including gliomas, acute myeloid leukemia (AML), and cholangiocarcinoma. These mutations result in a neomorphic enzyme activity that converts alpha-ketoglutarate to 2-hydroxyglutarate (2-HG), an oncometabolite. Accumulation of 2-HG leads to alterations in cellular metabolism and epigenetic dysregulation, promoting oncogenesis.
