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Symbol GP1BB contributors: npt/mct - updated : 24-02-2010
HGNC name glycoprotein Ib (platelet), beta polypeptide
HGNC id 4440
PROTEIN
PHYSICAL PROPERTIES Hydrophobic
STRUCTURE
motifs/domains
  • a N terminal signal peptide
  • one extracellular leucine-rich domain (LRD)
  • ectodomains of both GP1BB and GP9ubunits contain two leucine-rich repeats (LRR) and share high sequence similarity (Mo 2009)
  • a hydrophobic transmembrane domain near the C terminus
  • conjugated GlycoP
    mono polymer heteromer , dimer
    HOMOLOGY
    interspecies ortholog to murine Gp1bb
    homolog to C.elegans ZK994.4
    Homologene
    FAMILY leucine-rich glycoprotein family
    CATEGORY adhesion , receptor membrane
    SUBCELLULAR LOCALIZATION     plasma membrane
    text type I membrane protein
    basic FUNCTION
  • involved in adhesion of a damaged vessel wall to the extra cellular matrix
  • constituting the receptor for von Willebrand factor (VWF) and mediating platelet adhesion in the arterial circulation
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS coagulation/hemostasis
    PATHWAY
    metabolism
    signaling
    a component
  • component of the GP1b complex (von Willebrand receptor) where it is linked to GP1BA through disulfide bonds and non covalently to GP5 and GP9
  • a heterodimeric transmembrane protein consisting of a disulfide-linked 140 kD alpha chain and 22 kD beta chain
  • INTERACTION
    DNA
    RNA
    small molecule
    protein interacting with GP9 (putative convex surface of the LRR domain in GP9 is sufficient, in the context of full-length subunit, to mediate its association with GP1BB) (Mo 2009)
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) GP1BB
    related resource Bernard-Soulier Syndrome database
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS