GM-CSFR α Protein

Quality Statement:
GM-CSF receptor alpha is a member of the cytokine family of receptors. GM-CSF receptor alpha is found in the pseudoautosomal region of the X and Y chromosomes. GM-CSF receptor alpha has different isoform, with some of the isoforms being membrane-bound and others being soluble. Soluble GM-CSF receptor alpha subunit is a soluble isoform of the GMR alpha that is believed to arise exclusively through alternative splicing of the GMR alpha gene product. The sGMR alpha mRNA is expressed in a variety of tissues, but it is not clear which cells are capable of secreting the protein. Hereditary pulmonary alveolar proteinosis (hPAP) is a rare disorder of pulmonary surfactant accumulation and hypoxemic respiratory failure caused by mutations in GM-CSF receptor alpha, which results in reduced GM-CSF-dependent pulmonary surfactant clearance by alveolar macrophages. While no pharmacologic therapy currently exists for hPAP, it was recently demonstrated that endotracheal instillation of wild-type or gene-corrected mononuclear phagocytes results in a significant and durable therapeutic efficacy in a validated murine model of hPAP.

Reference:
1、Prevost J M. et al. (2002) Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF) and Inflammatory Stimuli Up-Regulate Secretion of the Soluble GM-CSF Receptor in Human Monocytes: Evidence for Ectodomain Shedding of the Cell Surface GM-CSF Receptor α Subunit. J Immunol. 169(10): 5679-5688.2、Cao Y. et al. (2007) Angiogenesis modulates adipogenesis and obesity. J Clin Invest. 117(9): 2362-2368.3、Fleetwood A J. et al. (2005) Functions of Granulocyte-Macrophage Colony-Stimulating Factor. Crit Rev Immunol. 25(5): 405-428.