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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. Note: This description may include information from UniProtKB.
Protein type: EC; 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; external side of plasma membrane; integral component of plasma membrane; intrinsic component of plasma membrane; membrane; microvillus; 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 complex 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: adrenal gland development; 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; estrogen metabolic process; extracellular matrix organization; hemopoietic progenitor cell differentiation; in utero embryonic development; Leydig cell differentiation; lung development; luteinization; male genitalia development; MAPK cascade; metanephric glomerular capillary formation; negative regulation of platelet activation; negative regulation of platelet-derived growth factor receptor-beta signaling pathway; odontogenesis of dentine-containing teeth; palate development; 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
Reference #:  P16234 (UniProtKB)
Alt. Names/Synonyms: Alpha-type platelet-derived growth factor receptor; CD140 antigen-like family member A; CD140A; CD140a antigen; MGC74795; PDGF-R-alpha; PDGFR2; PDGFRA; PGFRA; platelet-derived growth factor receptor, alpha polypeptide; rearranged-in-hypereosinophilia-platelet derived growth factor receptor alpha fusion protein; RHEPDGFRA
Gene Symbols: PDGFRA
Molecular weight: 122,670 Da
Basal Isoelectric point: 5.05  Predict pI for various phosphorylation states
CST Pathways:  Tyrosine Kinases & Substrates
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