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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
DNA
TYPE anonymous DNA segment
STRUCTURE 13.73 kb     8 Exon(s)
10 Kb 5' upstream gene genomic sequence study
text structure including one alternative exon
MAPPING cloned Y linked   status provisional
RNA
TRANSCRIPTS type messenger
text with two alternatively spliced isoforms, an additional exon and an 22 AA insertion in the prepro-leader sequence (Fujimaki)
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
8 - 1485 - 400 - 1998 9578570
EXPRESSION
Type restricted
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularvesselartery   
Digestiveliver    
Urinarykidney    
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Connectiveadipose   
cells
SystemCellPubmedSpeciesStageRna symbol
Cardiovascularendothelial cell
cell lineage
cell lines
fluid/secretion plasma
at STAGE
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