PDGFRB

Platelet-Derived Growth Factor Receptor Beta (PDGFRB) is a cell surface receptor that belongs to the class III receptor tyrosine kinase family. It plays a crucial role in various cellular processes, including cell proliferation, cell differentiation, and angiogenesis. PDGFRB is encoded by the PDGFRB gene in humans.

Structure

PDGFRB is composed of five immunoglobulin-like domains in its extracellular region, a single hydrophobic transmembrane segment, and a cytoplasmic part that contains the tyrosine kinase domain. The receptor is activated upon binding of its ligands, which are members of the platelet-derived growth factor (PDGF) family. This binding induces dimerization of the receptor, leading to autophosphorylation of specific tyrosine residues in the cytoplasmic domain, which in turn activates its kinase activity.

Function

The primary function of PDGFRB is to mediate the biological effects of PDGFs, which are key factors in the regulation of cell growth, neovascularization, and tissue regeneration. PDGFRB signaling is implicated in the development and maintenance of blood vessels, making it a significant player in the process of angiogenesis. Moreover, it has roles in the development of various tissues, including the nervous system and the kidneys.

Clinical Significance

Alterations in PDGFRB signaling, including overexpression and mutations, have been associated with several human diseases. Aberrant PDGFRB signaling is implicated in the pathogenesis of various types of cancer, including glioblastoma, sarcoma, and leukemia. Furthermore, mutations in the PDGFRB gene can lead to a range of developmental disorders and diseases, such as familial infantile myofibromatosis and Penttinen syndrome.

PDGFRB is also a target for cancer therapy, with several tyrosine kinase inhibitors (TKIs) developed to inhibit its kinase activity. These inhibitors are used in the treatment of cancers where PDGFRB signaling is known to play a critical role in tumor growth and survival.

Research

Ongoing research is focused on further elucidating the role of PDGFRB in normal physiology and disease, as well as developing more effective therapies targeting PDGFRB signaling in various diseases. Studies are also exploring the role of PDGFRB in other pathological conditions such as fibrosis and atherosclerosis.