Figure 7
Overproduction of 5hmC and disruption of histone methylation may contribute to DIPG tumorigenesis. Normal neural development is controlled by histone and DNA methylation executed by enhancer of zeste homologue 2 (EZH2) methyltransferase, DNA methyltransferase (DNMT), Ten Eleven Translocation (TET), Thymine-DNA glycosylase (TDG), and base excision repair (BER). Dysregulation of the epigenome by H3F3A mutation/EZH2 inhibition and corresponding overproduction of 5hmC by TET in DIPG may promote DIPG tumorigenicity.