C/EBPα deregulation as a paradigm for leukemogenesis

Myeloid master regulator CCAAT enhancer-binding protein alpha (C/EBP alpha) is deregulated by multiple mechanisms in leukemia. Inhibition of C/EBP alpha function plays pivotal roles in leukemogenesis. While much is known about how C/EBP alpha orchestrates granulopoiesis, our understanding of molecular transformation events, the role(s) of cooperating mutations and clonal evolution during C/EBP alpha deregulation in leukemia remains elusive. In this review, we will summarize the latest research addressing these topics with special emphasis on CEBP. mutations. We conclude by describing emerging therapeutic strategies to restore C/EBP alpha function.