Abstract
Polyomavirus enhancer binding protein 2 (PEBP2), also called core binding factor(CBF), is a heterodimer composed of the α and the β subunits. Structural alterations of each of the two subunits generated by recurrent chromosome translocations/inversion are associated with acute myeloid leukemia or acute lymphoblastic leukemia. Chimeric proteins containing a part of either the α or the β subunits have a potential to affect the transcriptional regulation through the PEBP2/CBF site. Structure and function of PEBP2/CBF and possible mechanisms of leukemogenesis caused by the chimeric proteins are summarized.