Encyclopedia of Medical Immunology

Living Edition
| Editors: Ian MacKay, Noel R. Rose

G-CSF Receptor Deficiency

  • Gesmar Rodrigues Silva SegundoEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-1-4614-9209-2_141-1
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Introduction

Granulocyte colony-stimulating factor (G-CSF) is a hematopoietic cytokine that stimulates neutrophil production and hematopoietic stem cell mobilization by starting the dimerization of homodimeric granulocyte colony-stimulating factor receptor. G-CSF receptor (G-CSFR) is member of type I cytokine receptor superfamily, encoded by CSF3R. G-CSFR is a transmembrane protein consisting of 813 amino acids: 604 as an extracellular domain, 26 in the transmembrane domain, and 184 in the intracellular cytoplasmic domain. The extracellular domain of the receptor contains an immunoglobulin-like (Ig-like) domain, a cytokine receptor homology domain (CRH domains), and three fibronectins type III (FN III) domains, and four highly conserved cysteine residues and a WSXWS motif of the CRH domains are required for ligand binding and receptor activation. The cytoplasmic region has no intrinsic kinase activity, but the receptor suffers a conformational change leading to the activation of...

Keywords

Granulocyte Colony-stimulating Factor (G-CSF) Severe Congenital Neutropenia (SCN) Chronic Neutrophilic Leukemia CSF3R Mutations Cytokine Receptor Homology Domain 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Pediatrics Allergy and ImmunologyUniversidade Federal de UberlandiaUberlandiaBrazil

Section editors and affiliations

  • Antonio Condino-Neto
    • 1
  1. 1.Institute of Biomedical SciencesUniversity of São PauloSão PauloBrasil