The role of the granulocyte colony-stimulating factor receptor (G-CSF-R) in disease
Ward, Alister 2007, The role of the granulocyte colony-stimulating factor receptor (G-CSF-R) in disease, Frontiers in bioscience, vol. 12, no. 2, pp. 608-618.
Title
The role of the granulocyte colony-stimulating factor receptor (G-CSF-R) in disease
Granulocyte colony-stimulating factor (G-CSF) is a key regulator of granulopoiesis via stimulation of a specific cell-surface receptor, the G-CSF-R, found on hematopoietic progenitor cells as well as neutrophilic granulocytes. It is perhaps not surprising, therefore, that mutations of the G-CSF-R has been implicated in several clinical settings that affect granulocytic differentiation, particularly severe congenital neutropenia, myelodysplastic syndrome and acute myeloid leukemia. However, other studies suggest that signalling via the G-CSF-R is also involved in a range of other malignancies. This review focuses on the molecular mechanisms through which the G-CSF-R contributes to disease.
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TABLE OF CONTENTS 1. Abstract 2. Introduction 2.1. G-CSF and its receptor 2.2. Neutropenia and other relevant disorders 2.3. G-CSF therapy 3. Direct role of G-CSF-R mutations in myeloid disorders 3.1. "Hyperresponsive" intracellular truncations 3.1.1. Clinical details 3.1.2. Mouse models 3.1.3. Molecular mechanisms 3.2. "Crippling" extracellular mutants 3.3. "Activating" transmembrane mutants 3.4. Other mutants 4. Indirect involvement of the G-CSF-R in disease 5. Conclusions 6. Acknowledgments 7. References (http://www.bioscience.org/current/vol12.htm)