Wilms tumor 1 mutations in the pathogenesis of acute myeloid leukemia

Wilms tumor 1 (WT1) has long been implicated in acute myeloid leukemia. It has been described to be both over-expressed and mutated in different forms of acute myeloid leukemia, and overexpression has been reported to play a prognostic role in this disease. However, the precise mechanism through which WT1 may play a role in leukemogenesis has remained elusive. In recent years, new evidence has emerged that points towards a novel role of WT1 mutations in the deregulation of epigenetic programs in leukemic cells through its interaction with TET proteins. Herein we review the current status of the field and its therapeutic and prognostic implications in acute myeloid leukemia.