G-CSF Receptor Deficiency
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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...