EZH2 Mutations: Mutating the Epigenetic Machinery in Myeloid Malignancies

Two recent studies identified loss-of-function mutations in the histone H3 methyltransferase EZH2 in myelodysplastic syndromes and myeloproliferative neoplasms, provided further demonstration of mutations in epigenetic modifiers in myeloid malignancies, and suggest EZH2 functions as a tumor-suppressor gene in these malignancies rather than an oncogene as in some other malignancies.