GM-CSFR α His Tag Protein, Mouse

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.