In gliomas and leukemias, IDH mutations have been shown to initiate tumorigenesis by D-2HG acting as a competitive inhibitor of α-KG-dependent dioxygenases such as the TET family of 5-methylcytosine hydroxylases, JumonjiC domain-containing histone demethylases (JHDMs) and the prolyl hydroxylases (PHDs) [5,8]. This evidence concerns the gene IDH1 and central nervous system cancer.