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    PROS1 protein S (alpha) [ Homo sapiens ]

    Gene ID: 5627, updated on 12-May-2012

    Summary

    Official Symbol
    PROS1provided by HGNC
    Official Full Name
    protein S (alpha)provided by HGNC
    Primary source
    HGNC:9456
    See related
    Ensembl:ENSG00000184500; HPRD:01473; MIM:176880; Vega:OTTHUMG00000150354
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Homo sapiens
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
    Also known as
    PSA; PROS; PS21; PS22; PS23; PS24; PS25; THPH5; THPH6
    Summary
    This gene encodes a vitamin K-dependent plasma protein that functions as a cofactor for the anticoagulant protease, activated protein C (APC) to inhibit blood coagulation. It is found in plasma in both a free, functionally active form and also in an inactive form complexed with C4b-binding protein. Mutations in this gene result in autosomal dominant hereditary thrombophilia. An inactive pseudogene of this locus is located at an adjacent region on chromosome 3. [provided by RefSeq, Feb 2009]

    Genomic context

    Location :
    3q11.2
    Sequence :
    Chromosome: 3; NC_000003.11 (93591881..93692934, complement)
    See PROS1 in Epigenomics, MapViewer

    Chromosome 3 - NC_000003.11Genomic Context describing neighboring genes Neighboring gene EPH receptor A3 Neighboring gene protein S pseudogene (beta) Neighboring gene high mobility group nucleosome binding domain 1 pseudogene 7 Neighboring gene ADP-ribosylation factor-like 13B Neighboring gene syntaxin 19

    Genomic regions, transcripts, and products

    Bibliography

    GeneRIFs: Gene References Into Functions What's a GeneRIF?

    Interactions

    Products Interactant Other Gene Complex Source Pubs Description
    P07225 P04003 C4BPA    HPRD  PubMed  
    P07225 P20851 C4BPB    HPRD  PubMed  
    P07225 P00742 F10    HPRD  PubMed  
    P07225 P00734 F2    HPRD  PubMed  
    P07225 P12259 F5    HPRD  PubMed  
    P07225 P00451 F8    HPRD  PubMed  
    P07225 P04070 PROC    HPRD  PubMed  
    P07225 Q06418 TYRO3    HPRD  PubMed  
    BioGRID:111611 BioGRID:108452 F5    BioGRID  PubMed Reconstituted Complex 
    BioGRID:111611 BioGRID:108455 F8    BioGRID  PubMed Reconstituted Complex 

    General gene information

    Markers

    Homology

    Pathways from BioSystems

    • Cell surface interactions at the vascular wall, organism-specific biosystem (from REACTOME)
      Cell surface interactions at the vascular wall, organism-specific biosystemLeukocyte extravasation is a rigorously controlled process that guides white cell movement from the vascular lumen to sites of tissue inflammation. The powerful adhesive interactions that are require...
    • Common Pathway, organism-specific biosystem (from REACTOME)
      Common Pathway, organism-specific biosystemThe common pathway consists of the cascade of activation events leading from the formation of activated factor X to the formation of active thrombin, the cleavage of fibrinogen by thrombin, and the f...
    • Complement and Coagulation Cascades, organism-specific biosystem (from WikiPathways)
      Complement and Coagulation Cascades, organism-specific biosystemBlood coagulation is a series of coordinated and calcium-dependent proenzyme-to-serine protease conversions likely to be localized on the surfaces of activated cells in vivo. It culminates in the for...
    • Complement and coagulation cascades, organism-specific biosystem (from KEGG)
      Complement and coagulation cascades, organism-specific biosystemThe complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation:...
    • Complement and coagulation cascades, conserved biosystem (from KEGG)
      Complement and coagulation cascades, conserved biosystemThe complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation:...
    • Complement cascade, organism-specific biosystem (from REACTOME)
      Complement cascade, organism-specific biosystemThe complement system is a biochemical cascade, so named because it 'complements' the ability of antibodies to clear pathogens. It is part of the innate immune system. Complement system proteins circ...
    • Formation of Fibrin Clot (Clotting Cascade), organism-specific biosystem (from REACTOME)
      Formation of Fibrin Clot (Clotting Cascade), organism-specific biosystemThe formation of a fibrin clot at the site of an injury to the wall of a normal blood vessel is an essential part of the process to stop blood loss after vascular injury. The reactions that lead to ...
    • Gamma-carboxylation of protein precursors, organism-specific biosystem (from REACTOME)
      Gamma-carboxylation of protein precursors, organism-specific biosystemGamma-carboxylation of a cluster of glutamate residues near the amino termini of thrombin, factor VII, factor IX, factor X, protein C, protein S, protein Z, and Gas 6 is required for these proteins t...
    • Gamma-carboxylation, transport, and amino-terminal cleavage of proteins, organism-specific biosystem (from REACTOME)
      Gamma-carboxylation, transport, and amino-terminal cleavage of proteins, organism-specific biosystemA number of proteins, including eight required for normal blood clot formation and its regulation (Prothrombin (factor II), factor VII, factor IX, factor X, protein C, protein S, protein Z, and Gas6)...
    • Hemostasis, organism-specific biosystem (from REACTOME)
      Hemostasis, organism-specific biosystemHemostasis is a physiological response that culminates in the arrest of bleeding from an injured vessel. Under normal conditions the vascular endothelium supports vasodilation, inhibits platelet adhe...
    • Immune System, organism-specific biosystem (from REACTOME)
      Immune System, organism-specific biosystemHumans are exposed to millions of potential pathogens daily, through contact, ingestion, and inhalation. Our ability to avoid infection depends on the adaptive immune system and during the first crit...
    • Innate Immune System, organism-specific biosystem (from REACTOME)
      Innate Immune System, organism-specific biosystemInnate immunity encompases the nonspecific part of immunity tha are part of an individual's natural biologic makeup
    • Metabolism of proteins, organism-specific biosystem (from REACTOME)
      Metabolism of proteins, organism-specific biosystemProtein metabolism comprises the pathways of translation, post-translational modification and protein folding.
    • PTM: gamma carboxylation, hypusine formation and arylsulfatase activation, organism-specific biosystem (from REACTOME)
      PTM: gamma carboxylation, hypusine formation and arylsulfatase activation, organism-specific biosystemAfter translation, many newly formed proteins undergo further covalent modifications that alter their functional properties and that are essentially irreversible under physiological conditions in the...
    • Platelet activation, signaling and aggregation, organism-specific biosystem (from REACTOME)
      Platelet activation, signaling and aggregation, organism-specific biosystemPlatelet activation begins with the initial binding of adhesive ligands and of the excitatory platelet agonists (released or generated at the sites of vascular trauma) to cognate receptors on the pla...
    • Platelet degranulation, organism-specific biosystem (from REACTOME)
      Platelet degranulation, organism-specific biosystemPlatelets function as exocytotic cells, secreting a plethora of effector molecules at sites of vascular injury. Platelets contain a number of distinguishable storage granules including alpha granules...
    • Post-translational protein modification, organism-specific biosystem (from REACTOME)
      Post-translational protein modification, organism-specific biosystemAfter translation, many newly formed proteins undergo further covalent modifications that alter their functional properties and that are essentially irreversible under physiological conditions in the...
    • Regulation of Complement cascade, organism-specific biosystem (from REACTOME)
      Regulation of Complement cascade, organism-specific biosystemTwo inherent features of complement activation make its regulation very important: 1. There is an inherent positive feedback loop because the product of C3 activation forms part of an enzyme that cau...
    • Removal of aminoterminal propeptides from gamma-carboxylated proteins, organism-specific biosystem (from REACTOME)
      Removal of aminoterminal propeptides from gamma-carboxylated proteins, organism-specific biosystemFurin is an endopeptidase localized to the Golgi membrane that cleaves many proteins on the carboxyterminal side of the sequence motif Arg-[any residue]-(Lys or Arg)-Arg (Jones et al. 1995; Leduc et ...
    • Response to elevated platelet cytosolic Ca2+, organism-specific biosystem (from REACTOME)
      Response to elevated platelet cytosolic Ca2+, organism-specific biosystemActivation of phospholipase C enzymes results in the generation of second messengers of the phosphatidylinositol pathway. The events resulting from this pathway are a rise in intracellular calcium an...
    • Transport of gamma-carboxylated protein precursors from the endoplasmic reticulum to the Golgi apparatus, organism-specific biosystem (from REACTOME)
      Transport of gamma-carboxylated protein precursors from the endoplasmic reticulum to the Golgi apparatus, organism-specific biosystemThe details of the vesicle-mediated transport of proteins from the endoplasmic reticulum to the Golgi apparatus will be annotated in a future release of Reactome.

    Gene Ontology Provided by GOA

    Function Evidence Code Pubs
    calcium ion binding IEA
    Inferred from Electronic Annotation
    more info
     
    endopeptidase inhibitor activity TAS
    Traceable Author Statement
    more info
    PubMed 
    Process Evidence Code Pubs
    blood coagulation TAS
    Traceable Author Statement
    more info
     
    cellular protein metabolic process TAS
    Traceable Author Statement
    more info
     
    leukocyte migration TAS
    Traceable Author Statement
    more info
     
    negative regulation of endopeptidase activity TAS
    Traceable Author Statement
    more info
    PubMed 
    peptidyl-glutamic acid carboxylation TAS
    Traceable Author Statement
    more info
     
    platelet activation TAS
    Traceable Author Statement
    more info
     
    platelet degranulation TAS
    Traceable Author Statement
    more info
     
    post-translational protein modification TAS
    Traceable Author Statement
    more info
     
    proteolysis TAS
    Traceable Author Statement
    more info
     
    Component Evidence Code Pubs
    Golgi lumen TAS
    Traceable Author Statement
    more info
     
    Golgi membrane TAS
    Traceable Author Statement
    more info
     
    endoplasmic reticulum membrane TAS
    Traceable Author Statement
    more info
     
    extracellular region NAS
    Non-traceable Author Statement
    more info
    PubMed 
    extracellular region TAS
    Traceable Author Statement
    more info
     
    platelet alpha granule lumen TAS
    Traceable Author Statement
    more info
     

    General protein information

    Preferred Names
    vitamin K-dependent protein S
    Names
    vitamin K-dependent protein S
    protein Sa
    vitamin K-dependent plasma protein S

    NCBI Reference Sequences (RefSeq)

    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    Genomic

    1. NG_009813.1 RefSeqGene

      Range
      5001..106054
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. NM_000313.3NP_000304.2  vitamin K-dependent protein S preproprotein

      Status: REVIEWED

      Source sequence(s)
      AC144562, AI139337, BC015801, DB275740
      Consensus CDS
      CCDS2923.1
      UniProtKB/Swiss-Prot
      P07225
      Related
      ENSP00000377783, OTTHUMP00000197145, ENST00000394236, OTTHUMT00000317762
      Conserved Domains (5) summary
      cd00054
      Location:119155
      Blast Score: 86
      EGF_CA; Calcium-binding EGF-like domain, present in a large number of membrane-bound and extracellular (mostly animal) proteins. Many of these proteins require calcium for their biological function and calcium-binding sites have been found to be located at the ...
      cd00110
      Location:312455
      Blast Score: 236
      LamG; Laminin G domain; Laminin G-like domains are usually Ca++ mediated receptors that can have binding sites for steroids, beta1 integrins, heparin, sulfatides, fibulin-1, and alpha-dystroglycans. Proteins that contain LamG domains serve a variety of ...
      cl02449
      Location:2385
      Blast Score: 252
      Gla; Vitamin K-dependent carboxylation/gamma-carboxyglutamic (GLA) domain
      cl00102
      Location:509647
      Blast Score: 107
      LamG; Laminin G domain; Laminin G-like domains are usually Ca++ mediated receptors that can have binding sites for steroids, beta1 integrins, heparin, sulfatides, fibulin-1, and alpha-dystroglycans. Proteins that contain LamG domains serve a variety of ...
      cl09941
      Location:201241
      Blast Score: 104
      EGF_CA; Calcium-binding EGF-like domain, present in a large number of membrane-bound and extracellular (mostly animal) proteins. Many of these proteins require calcium for their biological function and calcium-binding sites have been found to be located at the ...

    RefSeqs of Annotated Genomes: Build 37.3

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCh37.p5 Primary Assembly

    Genomic

    1. NC_000003.11 Reference GRCh37.p5 Primary Assembly

      Range
      93591881..93692934, complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Alternate HuRef

    Genomic

    1. AC_000135.1 Alternate HuRef

      Range
      90954621..91055633, complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Related Sequences

    Nucleotide Protein
    Heading Accession and Version
    genomic AB083386.1 BAC54134.1
    genomic AB083387.1 BAC54135.1
    genomic AB083389.1 BAC54137.1
    genomic AB083390.1 BAC54138.1
    genomic AB083391.1 BAC54139.1
    genomic AB083392.1 BAC54140.1
    genomic AB083393.1 BAC54141.1
    genomic AB083394.1 BAC54142.1
    genomic AB083395.1 BAC54143.1
    genomic AB083396.1 BAC54144.1
    genomic AB083687.1 BAC21163.1
    genomic AB083688.1 BAC21164.1
    genomic AB084900.1 BAC54253.1
    genomic AB084901.1 BAC54254.1
    genomic AB084902.1 BAC54255.1
    genomic AB084903.1 BAC54256.1
    genomic AB084904.1 BAC54257.1
    genomic AB087994.1 BAC55115.1
    genomic AC117474.7 (1994..91857) None
    genomic AC144562.5 (87101..97985) None
    genomic AY308744.1 AAP45054.1
    genomic AY605182.1 AAT37717.1
    genomic CH471052.2 EAW79903.1
      EAW79904.1
      EAW79905.1
    genomic DQ453481.1 ABD96173.1
    genomic DQ453483.1 ABD96174.1
    genomic DQ453484.1 ABD96175.1
    genomic DQ902690.1 ABI93128.1
    genomic M57853.1 AAA60357.1
    genomic S81775.1 AAD14374.1
    genomic S81777.1 AAD14375.1
    mRNA AI139337.1 None
    mRNA AK292994.1 BAF85683.1
    mRNA AK303895.1 BAG64828.1
    mRNA BC015801.1 AAH15801.1
    mRNA BE677834.1 None
    mRNA BU688598.1 None
    mRNA DB275740.1 None
    mRNA M15036.1 AAA36479.1
    mRNA X12892.1 CAA31383.1
    mRNA Y00692.1 CAA68687.1
      CAA68688.1
    mRNA M14338.1 AAA60181.1
    other-genetic DQ892389.2 ABM83315.1
    other-genetic DQ895602.2 ABM86528.1
    Protein Accession Links
    GenPept Link UniProtKB Link
    P07225.1 GenPept UniProtKB/Swiss-Prot:P07225
    Q06F34 GenPept UniProtKB/TrEMBL:Q06F34
    Q06F35 GenPept UniProtKB/TrEMBL:Q06F35
    Q16441 GenPept UniProtKB/TrEMBL:Q16441
    Q16519 GenPept UniProtKB/TrEMBL:Q16519
    Q1W146 GenPept UniProtKB/TrEMBL:Q1W146
    Q1W147 GenPept UniProtKB/TrEMBL:Q1W147
    Q1W148 GenPept UniProtKB/TrEMBL:Q1W148
    Q6J1N0 GenPept UniProtKB/TrEMBL:Q6J1N0
    Q7KYZ1 GenPept UniProtKB/TrEMBL:Q7KYZ1
    Q86Z10 GenPept UniProtKB/TrEMBL:Q86Z10
    Q8IU87 GenPept UniProtKB/TrEMBL:Q8IU87
    Q8IVH1 GenPept UniProtKB/TrEMBL:Q8IVH1
    Q8IXC3 GenPept UniProtKB/TrEMBL:Q8IXC3
    Q8IXC4 GenPept UniProtKB/TrEMBL:Q8IXC4
    Q8IXC5 GenPept UniProtKB/TrEMBL:Q8IXC5
    Q8IXC6 GenPept UniProtKB/TrEMBL:Q8IXC6
    Q8IXC8 GenPept UniProtKB/TrEMBL:Q8IXC8
    Q8IXC9 GenPept UniProtKB/TrEMBL:Q8IXC9
    Q8IXD0 GenPept UniProtKB/TrEMBL:Q8IXD0
    Q8IXD1 GenPept UniProtKB/TrEMBL:Q8IXD1
    Q8IXD2 GenPept UniProtKB/TrEMBL:Q8IXD2
    Q8IXD4 GenPept UniProtKB/TrEMBL:Q8IXD4
    Q8IXD5 GenPept UniProtKB/TrEMBL:Q8IXD5
    Q8J010 GenPept UniProtKB/TrEMBL:Q8J010
    Q8J011 GenPept UniProtKB/TrEMBL:Q8J011
    Q9NSD0 GenPept UniProtKB/TrEMBL:Q9NSD0

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