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FLASH GENE
Symbol HABP2 contributors: mct - updated : 17-03-2012
HGNC name hyaluronan binding protein 2
HGNC id 4798
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • three epidermal growth factor (EGF)
  • a kringle domain
  • a serine protease domain
  • mono polymer heteromer , dimer
    isoforms Precursor circulates as a single-chain proenzyme (pro-PHBP), which is converted to an active, two-chain form through autoproteolysis
    HOMOLOGY
    interspecies homolog to murine Habp2
    Homologene
    FAMILY
  • peptidase S1 family
  • CATEGORY adhesion , enzyme
    SUBCELLULAR LOCALIZATION extracellular
    basic FUNCTION
  • glycosaminoglycan binding activity
  • can activate coagulation factor VII and prourokinase and release the vasoactive peptide bradykinin by cleaving kininogen
  • undergoes autoactivation in the presence of polyanionic macromolecules such as heparin and RNA
  • negatively regulates vascular integrity via activation of protease-activated receptor/RHOA/RHO kinase signaling
  • serine protease implicated in proteolysis under inflammatory conditions
  • circulating serine protease with high homology to fibrinolytic enzymes
  • important role in the regulation of the haemostasis system as well as fibroproliferative inflammatory processes
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • heterodimer composed by a 50kDa protein and a 17kDa protein joined by disulfide bonds
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • cleaves single chain urokinase-type plasminogen activator
  • Histone binds to pro-PHBP (histone-mediated pro-PHBP activation results in bradykinin formation, which is implicated in septic shock)
  • cell & other
    REGULATION
    activated by histone that promotes pro-PHBP autoactivation
    Other polyamine leakage from legions through cell death or tissue injury may account for physiologically relevant pro-HABP2 activation
    regulation of its activity is dependent on its EGF-3 domain and over-expression of active variants induces cell death (
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility to carotid stenosis and deep venous thrombosis (DVT)
    Variant & Polymorphism other
  • association of the 1601GA genotype with incidence and progression of carotid stenosis and deep venous thrombosis (DVT)
  • Candidate gene
    Marker
    Therapy target
    SystemTypeDisorderPubmed
    cardiovascularaquired 
    potentially useful therapeutic target for syndromes of increased vascular permeability
    immunologyinfectious 
    selective inhibitors of pro-PHBP activation would be useful tools to treat septic shock
    ANIMAL & CELL MODELS