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C/EBPα deregulation as a paradigm for leukemogenesis

Leukemia volume 31, pages 2279–2285 (2017)Cite this article

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Abstract

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

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Acknowledgements

JAP is the recipient of a Scholar Award from American Society of Hematology, Young Investigator Award from Alex's Lemonade Stand Foundation for Childhood Cancer and a Discovery Grant from Lauri Strauss Leukemia Foundation. This work was supported by grants to DGT (a STaR Investigator Award; a RCE Core grant; a Tier 3 RNA Biology Center grant MOE2014-T3-1-006 from the NRF and MOE, Singapore; and grants CA66996 and CA197697 from the NCI/NIH) and to GB (grants BE 2042/12-1, BE 2042/7-1 and GRK1591 from DFG, German Research Foundation; grants DJCLS R 11/17, 17R/2016, R15/18, R12/31 from Deutsche Jose Carreras Leukämie-Stiftung e.V. and grants 2013.153.1 and 2015.093.1 from Wilhelm Sander Stiftung).

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Authors and Affiliations

  1. Department of Molecular, Cell and Cancer Biology, University of Massachusetts Medical School, Worcester, MA, USA

    J A Pulikkan

  2. Cancer Science Institute, National University of Singapore, Singapore, Singapore

    D G Tenen

  3. Harvard Stem Cell Institute, Harvard Medical School, Boston, MA, USA

    D G Tenen

  4. Division of Hematology and Oncology, Leipzig University Hospital, Leipzig, Germany

    G Behre

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Correspondence to J A Pulikkan, D G Tenen or G Behre.

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Pulikkan, J., Tenen, D. & Behre, G. C/EBPα deregulation as a paradigm for leukemogenesis. Leukemia 31, 2279–2285 (2017). https://doi.org/10.1038/leu.2017.229

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