EZH1 and malignant peripheral nerve sheath tumor: As previously mentioned, these mutations are observed in as many as 70% of MPNST, and other components of the PRC2 complex (such as EZH1 and EXH2) have been shown to be elevated in MPNST, making them an appealing target for drug development.14,15,43 PRC2 inactivation results in loss of H3K27me3, and a subsequent increase in acetyl groups,87resulting in recruitment of BET proteins, such as BRD4, to the chromatin.