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Hematol Oncol Clin North Am. 2013 Feb;27(1):61-73, viii. doi: 10.1016/j.hoc.2012.10.002. Epub 2012 Oct 31.

Granulocyte colony-stimulating factor receptor signaling: implications for G-CSF responses and leukemic progression in severe congenital neutropenia.

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1
Department of Hematology, Erasmus University Medical Center, Dr Molewaterplein 50 3015 GE, Rotterdam, The Netherlands. i.touw@erasmusmc.nl

Abstract

Following activation by their cognate ligands, cytokine receptors undergo intracellular routing toward lysosomes, where they are degraded. This review focuses on the signaling function of the G-CSFR in relation to the dynamics of endosomal routing of the G-CSFR. Mechanisms involving receptor lysine ubiquitination and redox-controlled phosphatase activities are discussed. Specific attention is paid to the consequences of G-CSFR mutations, acquired in patients with severe congenital neutropenias who receive G-CSF therapy, particularly in the context of leukemic transformation, a major clinical complication of the disease.

PMID:
23351988
DOI:
10.1016/j.hoc.2012.10.002
[Indexed for MEDLINE]
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