CSF2RA
Description
The CSF2RA (colony stimulating factor 2 receptor subunit alpha) is a protein-coding gene located on chromosome X|Y.
The granulocyte-macrophage colony-stimulating factor receptor, also known as CD116 (Cluster of Differentiation 116), is a receptor for granulocyte-macrophage colony-stimulating factor, which stimulates the production of white blood cells. In contrast to M-CSF and G-CSF which are lineage specific, GM-CSF and its receptor play a role in earlier stages of development. The receptor is primarily located on neutrophils, eosinophils and monocytes/macrophages, it is also on CD34+ progenitor cells (myeloblasts) and precursors for erythroid and megakaryocytic lineages, but only in the beginning of their development. It is associated with Surfactant metabolism dysfunction type 4.
== Structure == The granulocyte-macrophage colony-stimulating factor receptor is a heterodimer composed of at least two different subunits; an α chain, and a β chain which is also present in the receptors for IL-3 and IL-5. The α subunit contains a binding site for granulocyte macrophage colony-stimulating factor, but associates with the ligand only with low affinity. The β chain is involved in signal transduction and formation of high affinity receptor complex together with α chain. Furthermore association of the α and β subunits results in receptor activation.
=== α chain === Gene for α chain is in pseudoautosomal region (PAR) of X and Y chromosomes at the very tip of the chromosomes, near telomere regions and also genes encoding IL-3α with which they share some similarities. Along the gene are several transcription regulatory binding sites with common binding motifs for such transcription factors as GATA, C/EBP or NF-κB. α chain is 80kDa type I transmembrane protein composed of 3 domains: extracellular, transmembrane and cytoplasmic.
CSF2RA is also known as CD116, CDw116, CSF2R, CSF2RAX, CSF2RAY, CSF2RX, CSF2RY, GM-CSF-R-alpha, GMCSFR, GMCSFR-alpha, GMR, GMR-alpha, SMDP4, alphaGMR.
