PDGFRB: a receptor tyrosine kinase of the PDGFR family that binds members of the platelet-derived growth factor family. The identity of the growth factor bound determines whether the functional receptor is a homodimer or a heterodimer, composed of both PDGFR-alpha and -beta. Ligand binding induces receptor dimerization and autophosphorylation, thereby recruiting SH2-containing proteins including Grb2, Src, GAP, PTPN11, PI3 kinase, PLC-gamma and Nck. Regulates cell growth, actin reorganization, migration and differentiation. A variety of myeloproliferative disorders and cancers result from translocations that activate PDGFRbeta by fusion with proteins such as TEL/ETV6, H2, CEV14/TRP11, rabaptin 5, and huntington interacting protein 1. Gleevec treatment of TEL fusions has been successful. Overexpressed in metastatic medulloblastoma. Inhibitors: Gleevec, Sutent.
Protein type: EC 2.7.10.1; Kinase, protein; Membrane protein, integral; Oncoprotein; PDGFR family; Protein kinase, TK; Protein kinase, tyrosine (receptor); TK group
Chromosomal Location of Human Ortholog: 5q32
Cellular Component: apical plasma membrane; cell surface; cytoplasm; cytoplasmic vesicle; extracellular exosome; focal adhesion; integral component of membrane; intrinsic component of plasma membrane; lysosomal lumen; membrane; nucleus; plasma membrane
Molecular Function: ATP binding; enzyme binding; phosphatidylinositol-4,5-bisphosphate 3-kinase activity; phosphoinositide 3-kinase binding; platelet activating factor receptor activity; platelet-derived growth factor beta-receptor activity; platelet-derived growth factor binding; platelet-derived growth factor receptor binding; platelet-derived growth factor-activated receptor activity; protein binding; protein kinase binding; protein tyrosine kinase activity; Ras guanyl-nucleotide exchange factor activity; receptor binding; vascular endothelial growth factor binding
Biological Process: aging; cardiac myofibril assembly; cell migration; G-protein coupled receptor signaling pathway; glycosaminoglycan biosynthetic process; inner ear development; male gonad development; MAPK cascade; metanephric comma-shaped body morphogenesis; metanephric glomerular capillary formation; metanephric glomerular mesangial cell proliferation involved in metanephros development; metanephric mesenchyme development; metanephric S-shaped body morphogenesis; negative regulation of apoptosis; negative regulation of platelet-derived growth factor receptor-beta signaling pathway; peptidyl-tyrosine phosphorylation; phosphatidylinositol metabolic process; phosphatidylinositol phosphorylation; phosphoinositide-mediated signaling; platelet-derived growth factor receptor signaling pathway; positive regulation of apoptosis; positive regulation of calcium ion import; positive regulation of cell migration; positive regulation of cell proliferation; positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathway; positive regulation of chemotaxis; positive regulation of collagen biosynthetic process; positive regulation of DNA biosynthetic process; positive regulation of ERK1 and ERK2 cascade; positive regulation of fibroblast proliferation; positive regulation of hepatic stellate cell activation; positive regulation of MAP kinase activity; positive regulation of mitotic nuclear division; positive regulation of phosphatidylinositol 3-kinase activity; positive regulation of phosphoinositide 3-kinase cascade; positive regulation of phospholipase C activity; positive regulation of phosphoprotein phosphatase activity; positive regulation of protein kinase B signaling; positive regulation of reactive oxygen species metabolic process; positive regulation of Rho protein signal transduction; positive regulation of smooth muscle cell migration; positive regulation of smooth muscle cell proliferation; protein autophosphorylation; regulation of actin cytoskeleton organization; response to estradiol; response to estrogen; response to hydrogen peroxide; response to hyperoxia; response to retinoic acid; response to toxin; signal transduction; smooth muscle cell chemotaxis; wound healing
Disease: Basal Ganglia Calcification, Idiopathic, 1; Basal Ganglia Calcification, Idiopathic, 4; Kosaki Overgrowth Syndrome; Myeloproliferative Disorder, Chronic, With Eosinophilia; Myofibromatosis, Infantile, 1; Premature Aging Syndrome, Penttinen Type
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