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FLASH GENE
Symbol PDGFRB contributors: mct/pgu - updated : 15-07-2016
HGNC name platelet-derived growth factor receptor, beta polypeptide
HGNC id 8804
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
23 - 5718 - 1106 - 1995 7641887
EXPRESSION
Rna function mRNA for PDGFRB only in 25p100 of the oocytes
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Endocrinepancreas     Homo sapiens
Nervousbrain   highly Homo sapiens
Reproductivefemale systemovary  highly
Respiratoryrespiratory tractlarynx  highly
Skin/Tegumentskin     Homo sapiensFetal
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Connectivebone  highly
Connectiveinterstitialdermis   Homo sapiensFetal
Muscularsmoothvessel   Homo sapiens
cells
SystemCellPubmedSpeciesStageRna symbol
Endocrineislet cell (alpha,beta...) Homo sapiens
Nervousastrocyte Homo sapiens
Nervousneuron Homo sapiens
not specificfibroblast Homo sapiensFetal
cell lineage
cell lines
fluid/secretion
at STAGE
physiological period growth/childhood
Text
  • PDGFRB levels were markedly reduced in islet beta-cells at 6 weeks and 6 months of age, compared to neonatal islets
  • PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • five extracellular Ig-like motifs
  • a transmembrane segment (1TM)
  • a cytoplasmic split tyrosine kinase domain
  • mono polymer homomer , heteromer , dimer
    HOMOLOGY
    Homologene
    FAMILY
  • protein kinase superfamily
  • Tyr protein kinase family
  • CSF-1/PDGF receptor subfamily
  • CATEGORY signaling cytokine growth factor , receptor
    SUBCELLULAR LOCALIZATION     plasma membrane
        intracellular
    intracellular,cytoplasm,organelle,lumen
    intracellular,cytoplasm,organelle,lysosome
    intracellular,cytoplasm,cytosolic,vesicle
    intracellular,nucleus
    basic FUNCTION
  • receptor tyrosine kinase, class III, phosphorylating Tyr residues at the C-terminus of PTPN11 creating a binding site for the SH2 domain of GRB2
  • mitogen for mesenchyme-derived cells, including fibroblasts and smooth muscle
  • phosphorylates GRK2 tyrosyl residues and thereby activates GRK2, which then serine-phosphorylates and desensitizes the PDGFRbeta
  • PDGFRB signaling is crucial for neuroprotection, endogenous tissue repair, and functional recovery after stroke by targeting neurons,pericyte/vascular smooth muscle cells, and astrocytes
  • required for the development of mesenchymal cell types, and plays a diverse role in the function of fibroblasts in tissue homeostasis and regeneration
  • PDGFRB-mediated signaling plays a key role in morphine tolerance
  • has an essential role in vascular progenitor cell signaling (pMID: 31031011)
  • CELLULAR PROCESS cell life, proliferation/growth
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
  • PDGFRB-AKT1 signaling in brain pericytes may play various important roles leading to neuroprotection after ischemic stroke
  • a component
  • homodimerizing and heterodimerizing with PDGFRA
  • INTERACTION
    DNA
    RNA
    small molecule nucleotide,
  • ATP
  • protein
  • binds specifically to PDGFRA and has a tyrosine-protein kinase activity
  • interacting with NRP1 (influences ligand-induced PDGFRB homodimer signalling)
  • important interactive relationship between SLC6A4 and the PDGFRB in the production of pulmonary artery smooth muscle cell proliferation triggered by PDGF that may be important in pulmonary hypertension (PH)
  • LRP6 forms a complex with PDGFRB and promotes its degradation
  • SH2B3 is a negative regulator of PDGFR signaling
  • in aortic smooth muscle cells, TGM2 specifically amplifies the activation of PDGFRB and its multiple downstream signaling targets in response to PDGF-BB
  • ETV6-PDGFRB antagonizes the effects of ETV6 and IRF8 on PTPN13 transcription
  • PDGFRB/MAPK1 signalling pathway triggered by FGF2 plays an important role in the proliferation and migration of endothelial progenitor cells
  • involved in angiogenesis and in the regulation of inorganic phosphate (Pi) transport in vascular smooth
  • muscle cells via SLC20A1
  • PDGFRB expression was upregulated by NOTCH3 ligand induction or by activated forms of the NOTCH3 receptor
  • affects the intracellular sorting of activated PDGFRB and the migration, proliferation and tumorigenicity of cells stimulated by PDGF
  • cell & other
    REGULATION
    activated by constitutively activated through amino-acid(s) substitution in a WW-like domain (phosphoserine- or phosphothreonine binding)
    repressed by ZNF24
    Other phosphorylated by PTPN11 controlling Ras GAP recruitement and Ras MAP kinase signaling in the heterodimeric configuration of PDGF receptors
    ligand-mediated stabilization of specific G-quadruplex structures in the promoter can modulate its transcription
    ASSOCIATED DISORDERS
    corresponding disease(s) IMF1 , BGCI4 , PENTT , POGS
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral       gain of function
    in clear cell renal carcinoma with favourable outcome
    tumoral fusion translocation   protein chimeric
    t(5;12)(q33;p13) in myelodysplasia, myeloproliferative disorders (MPS), chronic myelocytic leukemia (CMLL), with fusion 5' - ETV66 - PDGFRB - 3'
    constitutional germinal mutation      
    gain of function mutation in the activation loop deregulates its kinase activity
    tumoral fusion translocation    
    somatic traslocations in various hematological disorders resulting in PDGFRB/D10S170 fusion gene, PDGFRB/RABPT5 fusion gene, PDGFRB/HIP1 fusion gene and other partners genes
    tumoral fusion      
    with MYO18A in an eosinophilia-associated atypical myeloproliferative neoplasm with a t(5;17)(q33-34;q11.2)
    tumoral fusion      
    to SPTBN1 in imatinib-responsive atypical myeloproliferative disorders
    constitutional somatic mutation      
    in intracerebral fusiform aneurysms
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • PDGFRB inhibition selectively eliminates morphine tolerance in rats