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FLASH GENE
Symbol PROZ contributors: mct - updated : 25-05-2011
HGNC name protein Z, vitamin K-dependent plasma glycoprotein
HGNC id 9460
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • at the N terminus, a GLA domain
  • two EGF domains
  • a hinge region
  • a homolog of the catalytic B chain of serine proteases
  • a peptidase S1 domain
  • conjugated GlycoP
    HOMOLOGY
    Homologene
    FAMILY
    CATEGORY structural protein
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    intracellular,cytoplasm,organelle,lumen
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    intracellular,cytoplasm,organelle,Golgi
    text presence of PROZ near the nucleus and a possible expression at the membrane
    basic FUNCTION
  • vitamin K-dependent protein isolated from human plasma, and acting as a cofactor for a serpin, called protein Z-dependent protease inhibitor (ZPI)
  • forming a Ca2 ion-dependent complex with activated F10 and a PROZ dependent protein inhibitor
  • involved in hemostasis by binding thrombin and promoting its association with phospholipid vesicles
  • being an important factor in the pathogenesis of ischemic stroke due to large-artery atherothromboembolism or an acute phase reactant
  • normal increase of protein Z during pregnancy may balance the increase of clotting factors to protect pregnant women from thrombosis
  • cofactor of PZ dependent inhibitor (ZPI) that inhibits activated coagulation factor X
  • role for protein Z deficiency in thrombotic diseases, including arterial thrombosis, pregnancy complications and venous thromboembolism
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS coagulation/hemostasis
    PATHWAY
    metabolism
    signaling
    a component regulation of coagulation
    INTERACTION
    DNA
    RNA
    small molecule
    protein binding to thrombin and promoting its association with phospholipid vesicles
    cell & other
    REGULATION
    Other vitamin K-dependent
    ASSOCIATED DISORDERS
    corresponding disease(s) PROZ
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    is a risk factor for retinal vessel occlusion
    constitutional     --low  
    associated with an increased risk of thrombosis
    constitutional     --low  
    in chronic kidney disease
    constitutional        
    high rate of protein Z deficiency is observed in patients with preeclampsia and fetal demise (
    Susceptibility
  • to recurrent early pregnancy loss (RPL)
  • to premature myocardial infarction (Minor role) (
  • Variant & Polymorphism other
  • isolated presence of the intron F 79A allele as well as the combination with known thrombophilic risk factors was protective against RPL between the 8th and 12th weeks of gestation
  • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS