2017-04-13
Expression and regulation of C/EBPα in normal myelopoiesis and in malignant transformation.
Publication
Publication
Blood , Volume 129 - Issue 15 p. 2083- 2091
One of the most studied transcription
factors in hematopoiesis is the leucine
zipper CCAAT-enhancer binding proteina
(C/EBPα), which is mainly involved in cell
fate decisions for myeloid differentiation.
Its involvement in acute myeloid leukemia
(AML) is diverse, with patients frequently
exhibiting mutations, deregulation of gene
expression, or alterations in the function of
C/EBPα. In this review, we emphasize the
importance of C/EBPα for neutrophil maturation,
its role in myeloid priming of hematopoietic
stem and progenitor cells, and its
indispensable requirement for AML development.
We discuss that mutations in the
open reading frame of CEBPA lead to an
altered C/EBPa function, affecting the expression
of downstream genes and consequently
deregulating myelopoiesis. The
emerging transcriptional mechanisms of
CEBPA are discussed based on recent
studies. Novel insights on howthese mechanisms
may be deregulated by oncoproteins
or mutations/variants in CEBPA
enhancers are suggested in principal to
reveal novel mechanisms of how CEBPA is
deregulated at the transcriptional level.
Additional Metadata | |
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This work was supported by the Dutch Cancer Foundation “Konining Wilhelmina Fonds” and the Ladt Tata Memorial Trust Foundation. | |
doi.org/10.1182/blood-2016-09- 687822, hdl.handle.net/1765/106478 | |
Blood | |
Organisation | Department of Hematology |
Avellino, R., & Delwel, R. (2017). Expression and regulation of C/EBPα in normal myelopoiesis and in malignant transformation. Blood, 129(15), 2083–2091. doi:10.1182/blood-2016-09- 687822 |