Recombinant Human Cytokine receptor common subunit beta (CSF2RB), partial (Active)

Function

Cell surface receptor that plays a role in immune response and controls the production and differentiation of hematopoietic progenitor cells into lineage-restricted cells. Acts by forming an heterodimeric receptor through interaction with different partners such as IL3RA, IL5RA or CSF2RA (PubMed:1495999). In turn, participates in various signaling pathways including interleukin-3, interleukin-5 and granulocyte-macrophage colony-stimulating factor/CSF2 pathways. In unstimulated conditions, interacts constitutively with JAK1 and ligand binding leads to JAK1 stimulation and subsequent activation of the JAK-STAT pathway (PubMed:9516124)

Subcellular Location

Membrane (Single-pass type I membrane protein)

Disease Association

Pulmonary surfactant metabolism dysfunction 5 (SMDP5) : A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress. [The disease is caused by variants affecting the gene represented in this entry]

Subunit

Heterodimer of an alpha and a beta subunit. The beta subunit is common to the IL3, IL5 and GM-CSF receptors (PubMed:1495999). The signaling GM-CSF receptor complex is a dodecamer of two head-to-head hexamers of two alpha, two beta, and two ligand subunits. Interacts with TMEM102; this interaction occurs preferentially in the absence of CSF2. Interacts with FCER1G; this interaction is direct. Interacts with LYN. Interacts with JAK1 (PubMed:9516124)

• Hayashida K. et al. Molecular cloning of a second subunit of the receptor for human granulocyte-macrophage colony-stimulating factor (GM-CSF): reconstitution of a high-affinity GM-CSF receptor. — Proc. Natl. Acad. Sci. U.S.A. (1990)
• Kitamura T. et al. PubMed: CI-CQ3UTBH3EH0RN (FEB-)
• Collins J.E. et al. A genome annotation-driven approach to cloning the human ORFeome. — Genome Biol. (2004)
• Dunham I. et al. The DNA sequence of human chromosome 22. — Nature (1999)
• Mural R.J. et al. PubMed: CI-5GBDQ6B103N1E (JUL-)
• Kao C.J. et al. CBAP interacts with the un-liganded common beta-subunit of the GM-CSF/IL-3/IL-5 receptor and induces apoptosis via mitochondrial dysfunction. — Oncogene (2008)
• Tanaka T. et al. Adult-onset hereditary pulmonary alveolar proteinosis caused by a single-base deletion in CSF2RB. — J. Med. Genet. (2011)
• Tavernier J. et al. Molecular basis of the membrane-anchored and two soluble isoforms of the human interleukin 5 receptor alpha subunit. — Proc. Natl. Acad. Sci. U.S.A. (1992)