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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; integral to plasma membrane; intrinsic to plasma membrane; membrane; nucleus; plasma membrane; protein complex
Molecular Function: 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 receptor activity
Biological Process: cardiac myofibril assembly; cell activation; elevation of cytosolic calcium ion concentration; embryonic cranial skeleton morphogenesis; embryonic digestive tract morphogenesis; embryonic skeletal morphogenesis; luteinization; MAPKKK cascade; peptidyl-tyrosine phosphorylation; phosphoinositide-mediated signaling; platelet-derived growth factor receptor signaling pathway; positive regulation of cell migration; positive regulation of cell proliferation; positive regulation of DNA replication; positive regulation of fibroblast proliferation; positive regulation of phosphoinositide 3-kinase activity; positive regulation of phosphoinositide 3-kinase cascade; protein amino acid autophosphorylation; regulation of chemotaxis; regulation of phosphoinositide 3-kinase cascade; 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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