Platelet-Derived Growth Factor Soluble Receptor Alpha (PDGFsR-alpha) ELISA Kit for Human

This gene encodes a cell surface tyrosine kinase receptor for members of the platelet-derived growth factor family. These growth factors are mitogens for cells of mesenchymal origin. The identity of the growth factor bound to a receptor monomer determines whether the functional receptor is a homodimer or a heterodimer, composed of both platelet-derived growth factor receptor alpha and beta polypeptides. Studies suggest that this gene plays a role in organ development, wound healing, and tumor progression. Mutations in this gene have been associated with idiopathic hypereosinophilic syndrome, somatic and familial gastrointestinal stromal tumors, and a variety of other cancers. [provided by RefSeq, Mar 2012]

PDGFRA: 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. Particularly important for kidney development since mice heterozygous for the receptor exhibit defective kidney phenotypes. Chromosomal rearrangments activate PDGFRalpha by fusion to BCR, causing atypical chronic myelogenous leukemia (CML), and to FIP1L1, causing idiopathic hypereosinophilic syndrome. Activating point mutations cause a minority of gastrointestinal stromal tumors (GIST). Promoter polymorphisms linked to neural tube defects including spina bifida, verified by mouse mutant model. Inhibitors: Gleevec, Sutent. OMIM: Two alternatively-spliced isoforms have been described.

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: 4q12

Cellular Component: cell junction; cytoplasm; integral component of plasma membrane; intrinsic component of plasma membrane; membrane; nucleus; plasma membrane; protein complex

Molecular Function: ATP binding; phosphatidylinositol-4,5-bisphosphate 3-kinase activity; platelet-derived growth factor alpha-receptor activity; platelet-derived growth factor binding; platelet-derived growth factor receptor binding; protein binding; protein homodimerization activity; protein kinase activity; Ras guanyl-nucleotide exchange factor activity; transmembrane receptor protein tyrosine kinase activity; vascular endothelial growth factor binding; vascular endothelial growth factor-activated receptor activity

Biological Process: cardiac myofibril assembly; cell activation; cellular response to reactive oxygen species; elevation of cytosolic calcium ion concentration; embryonic cranial skeleton morphogenesis; embryonic digestive tract morphogenesis; embryonic skeletal morphogenesis; luteinization; MAPK cascade; metanephric glomerular capillary formation; negative regulation of platelet activation; negative regulation of platelet-derived growth factor receptor-beta signaling pathway; peptidyl-tyrosine phosphorylation; phosphatidylinositol phosphorylation; phosphoinositide-mediated signaling; platelet-derived growth factor receptor signaling pathway; 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 DNA replication; positive regulation of ERK1 and ERK2 cascade; positive regulation of fibroblast proliferation; positive regulation of phosphatidylinositol 3-kinase activity; positive regulation of phosphoinositide 3-kinase cascade; positive regulation of phospholipase C activity; positive regulation of protein kinase B signaling; protein autophosphorylation; regulation of actin cytoskeleton reorganization; regulation of chemotaxis; regulation of mesenchymal stem cell differentiation; viral process; wound healing

Disease: Gastrointestinal Stromal Tumor; Hypereosinophilic Syndrome, Idiopathic