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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
Corresponding disease
PROZ PROZ deficiency
Location 13q34      Physical location : 113.812.967 - 113.826.694
Synonym symbol(s) PZ
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
Physical map
LOC387947 13 LOC387947 LOC390426 13 similar to mKIAA0324 protein SOX1 13q34 SRY (sex determining region Y)-box 1 LOC387948 13 LOC387948 LOC122258 13q34 LOC122258 TUBGCP3 13q34 tubulin, gamma complex associated protein 3 LOC387949 13 LOC387949 ATP11A 13q34 ATPase, Class VI, type 11A LOC387950 13 LOC387950 MCF2L 13q34 MCF.2 cell line derived transforming sequence-like F7 13q34 coagulation factor VII (serum prothrombin conversion accelerator) F10 13q34 coagulation factor X PROZ 13q34 protein Z, vitamin K-dependent plasma glycoprotein FLJ11305 13q34 hypothetical protein FLJ11305 CUL4A 13q34-qter cullin 4A LAMP1 13q34 lysosomal-associated membrane protein 1 GRTP1 4q14 growth hormone regulated TBC protein 1 LOC387951 13 LOC387951 ADPRHL1 13q34 ADP-ribosylhydrolase like 1 C13orf17 13q34 chromosome 13 open reading frame 17 C13orf11 13q34 chromosome 13 open reading frame 11 TFDP1 13q34 transcription factor Dp-1 ATP4B 13q34 ATPase, H+/K+ exchanging, beta polypeptide RHOK 13q34 rhodopsin kinase LOC387952 13 hypothetical gene supported by AK126042; BC034570 GAS6 13q34 growth arrest-specific 6 MGC20579 13q34 hypothetical protein MGC20579 RASA3 13q34 RAS p21 protein activator 3 LOC390427 13 LOC390427 CDC16 13q34 CDC16 cell division cycle 16 homolog (S. cerevisiae) UPF3A 13q34 UPF3 regulator of nonsense transcripts homolog A (yeast) C13orf8 13q34 chromosome 13 open reading frame 8
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