Complexity of CEBPA dysregulation in human acute myeloid leukemia

Pabst, Thomas; Mueller, Beatrice U (2009). Complexity of CEBPA dysregulation in human acute myeloid leukemia. Clinical cancer research, 15(17), pp. 5303-7. Philadelphia, Pa.: American Association for Cancer Research 10.1158/1078-0432.CCR-08-2941

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The transcription factor CCAAT enhancer binding protein alpha (CEBPA) is crucial for normal development of granulocytes. Various mechanisms have been identified how CEBPA function is dysregulated in patients with acute myeloid leukemia (AML). In particular, dominant-negative mutations located either at the N- or the C terminus of the CEBPA gene are observed in roughly 10% of AML patients, either in the combination on separate alleles or as sole mutation. Clinically significant complexity exists among AML with CEBPA mutations, and patients with double CEBPA mutations seem to have a more favorable course of the disease than patients with a single mutation. In addition, myeloid precursor cells of healthy carriers with a single germ-line CEBPA mutation evolve to overt AML by acquiring a second sporadic CEBPA mutation. This review summarizes recent reports on dysregulation of CEBPA function at various levels in human AML and therapeutic concepts targeting correction of CEBPA activity. The currently available data are persuasive evidence that impaired CEBPA function contributes directly to the development of AML, whereas restoring CEBPA function represents a promising target for novel therapeutic strategies in AML.

Item Type:

Journal Article (Further Contribution)


04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Medical Oncology
04 Faculty of Medicine > Department of General Internal Medicine (DAIM) > Clinic of General Internal Medicine > Centre of Competence for General Internal Medicine

UniBE Contributor:

Pabst, Thomas and Müller, Beatrice Ursula




American Association for Cancer Research




Factscience Import

Date Deposited:

04 Oct 2013 15:11

Last Modified:

11 Jul 2018 17:05

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URI: (FactScience: 195880)

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