Colony stimulating factor 2 receptor alpha subunit (CSF2RA)

The Colony stimulating factor 2 receptor alpha subunit (CSF2RA), also known as CD116, is a transmembrane protein that serves as the ligand-specific component of the receptor for granulocyte-macrophage colony-stimulating factor (GM-CSF) (UniProt P15509). It binds GM-CSF with low affinity but forms a high-affinity signaling complex upon heterodimerization with the common beta subunit (CSF2RB), which is shared with IL-3 and IL-5 receptors (NCBI Gene 1438). This receptor is predominantly expressed on myeloid lineage cells, including macrophages, neutrophils, and eosinophils, where it triggers the JAK2-STAT5 signaling pathway to regulate cell survival, proliferation, and functional activation (StatPearls). In clinical medicine, CSF2RA is a critical driver of inflammatory pathology; for instance, its overactivation contributes to the progression of rheumatoid arthritis and other autoimmune conditions (Burmester et al., 2017, Annals of the Rheumatic Diseases). Conversely, genetic deficiencies or mutations in CSF2RA lead to hereditary pulmonary alveolar proteinosis (PAP), a condition characterized by the accumulation of surfactant in the lungs due to defective alveolar macrophage function (Trapnell et al., 2003, NEJM). Therapeutic targeting of CSF2RA primarily involves monoclonal antibodies like mavrilimumab, which acts as an antagonist to block GM-CSF-mediated inflammation in diseases like rheumatoid arthritis and giant cell arteritis (ClinicalTrials.gov). Additionally, recombinant GM-CSF analogs like sargramostim act as agonists to stimulate white blood cell production in patients with neutropenia or following bone marrow transplantation (FDA).

Other names

CD116GM-CSFR-alphaGranulocyte-macrophage colony-stimulating factor receptor subunit alphaGMR-alphaCSF2RCDw116

Antagonism of the alpha subunit to block GM-CSF binding and downstream signaling, or agonism via recombinant GM-CSF to stimulate myeloid cell production and activation.