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Aliases for PROS1 Gene

Aliases for PROS1 Gene

  • Protein S (Alpha) 2 3
  • THPH5 3 6
  • THPH6 3 6
  • PROS 3 4
  • Vitamin K-Dependent Plasma Protein S 3
  • Vitamin K-Dependent Protein S 3
  • Protein Sa 3
  • EC 3.4.21 63
  • PS21 3
  • PS22 3
  • PS23 3
  • PS24 3
  • PS25 3
  • PSA 3

External Ids for PROS1 Gene

Previous HGNC Symbols for PROS1 Gene

  • PROS

Previous GeneCards Identifiers for PROS1 Gene

  • GC03P090396
  • GC03M092806
  • GC03M090130
  • GC03M094912
  • GC03M095074
  • GC03M093591
  • GC03M090954

Summaries for PROS1 Gene

Entrez Gene Summary for PROS1 Gene

  • 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]

GeneCards Summary for PROS1 Gene

PROS1 (Protein S (Alpha)) is a Protein Coding gene. Diseases associated with PROS1 include thrombophilia due to protein s deficiency, autosomal dominant and thrombophilia due to protein s deficiency, autosomal recessive. Among its related pathways are Hemostasis and Formation of Fibrin Clot (Clotting Cascade). GO annotations related to this gene include calcium ion binding and endopeptidase inhibitor activity. An important paralog of this gene is SHBG.

UniProtKB/Swiss-Prot for PROS1 Gene

  • Anticoagulant plasma protein; it is a cofactor to activated protein C in the degradation of coagulation factors Va and VIIIa. It helps to prevent coagulation and stimulating fibrinolysis

Gene Wiki entry for PROS1 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for PROS1 Gene

Genomics for PROS1 Gene

Regulatory Elements for PROS1 Gene

Genomic Location for PROS1 Gene

93,873,033 bp from pter
93,974,090 bp from pter
101,058 bases
Minus strand

Genomic View for PROS1 Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for PROS1 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for PROS1 Gene

Proteins for PROS1 Gene

  • Protein details for PROS1 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Vitamin K-dependent protein S
    Protein Accession:
    Secondary Accessions:
    • A8KAC9
    • D3DN28
    • Q15518
    • Q7Z715
    • Q9UCZ8

    Protein attributes for PROS1 Gene

    676 amino acids
    Molecular mass:
    75123 Da
    Quaternary structure:
    No Data Available
    • Sequence=AAP45054.1; Type=Erroneous gene model prediction; Evidence={ECO:0000305};

    Three dimensional structures from OCA and Proteopedia for PROS1 Gene

neXtProt entry for PROS1 Gene

Proteomics data for PROS1 Gene at MOPED

Selected DME Specific Peptides for PROS1 Gene

Post-translational modifications for PROS1 Gene

  • The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
  • Glycosylation at Asn499, Asn509, and Asn530
  • Modification sites at PhosphoSitePlus

Other Protein References for PROS1 Gene

ENSEMBL proteins:
Reactome Protein details:
REFSEQ proteins:

Domains for PROS1 Gene

Graphical View of Domain Structure for InterPro Entry



  • P07225
  • Contains 4 EGF-like domains.
  • Contains 1 Gla (gamma-carboxy-glutamate) domain.
  • Contains 2 laminin G-like domains.
genes like me logo Genes that share domains with PROS1: view

No data available for Gene Families for PROS1 Gene

Function for PROS1 Gene

Molecular function for PROS1 Gene

GENATLAS Biochemistry: protein S,alpha coagulation factor (cofactor of proC),vitamin K-dependent,ligand for TYRO3,AXL family of receptor tyrosine kinase
UniProtKB/Swiss-Prot Function: Anticoagulant plasma protein; it is a cofactor to activated protein C in the degradation of coagulation factors Va and VIIIa. It helps to prevent coagulation and stimulating fibrinolysis

Enzyme Numbers (IUBMB) for PROS1 Gene

Gene Ontology (GO) - Molecular Function for PROS1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004866 endopeptidase inhibitor activity TAS 8146182
GO:0005509 calcium ion binding IEA --
GO:0005515 protein binding --
genes like me logo Genes that share ontologies with PROS1: view
genes like me logo Genes that share phenotypes with PROS1: view

Animal Models for PROS1 Gene

MGI Knock Outs for PROS1:

miRNA for PROS1 Gene

miRTarBase miRNAs that target PROS1

No data available for Transcription Factor Targeting and HOMER Transcription for PROS1 Gene

Localization for PROS1 Gene

Subcellular locations from UniProtKB/Swiss-Prot for PROS1 Gene


Subcellular locations from

Jensen Localization Image for PROS1 Gene COMPARTMENTS Subcellular localization image for PROS1 gene
Compartment Confidence
extracellular 5
endoplasmic reticulum 4
golgi apparatus 4
cytoskeleton 2
plasma membrane 2
lysosome 1
mitochondrion 1
nucleus 1

Gene Ontology (GO) - Cellular Components for PROS1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000139 Golgi membrane TAS --
GO:0005576 extracellular region TAS --
GO:0005615 extracellular space IDA 14607961
GO:0005789 endoplasmic reticulum membrane TAS --
GO:0005796 Golgi lumen TAS --
genes like me logo Genes that share ontologies with PROS1: view

Pathways for PROS1 Gene

genes like me logo Genes that share pathways with PROS1: view

Gene Ontology (GO) - Biological Process for PROS1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0002576 platelet degranulation TAS --
GO:0006508 proteolysis TAS --
GO:0007596 blood coagulation TAS --
GO:0010951 negative regulation of endopeptidase activity TAS 8146182
GO:0017187 peptidyl-glutamic acid carboxylation TAS --
genes like me logo Genes that share ontologies with PROS1: view

Compounds for PROS1 Gene

(1) HMDB Compounds for PROS1 Gene

Compound Synonyms Cas Number PubMed IDs
  • Vitamin K3: 1,4-Dihydro-1,4-dioxo-2-methylnaphthalene

(3) Drugbank Compounds for PROS1 Gene

Compound Synonyms Cas Number Type Actions PubMed IDs
Drotrecogin alfa
  • Anticoagulant protein C
60202-16-6 target
  • 2-Methyl-1,4-Naphthalenedione
58-27-5 target activator
Sodium Tetradecyl Sulfate
  • Monotetradecylsulfate sodium salt
1191-50-0 target inhibitor

(29) Novoseek inferred chemical compound relationships for PROS1 Gene

Compound -log(P) Hits PubMed IDs
estramustine phosphate 72.1 1
gamma-carboxyglutamic acid 67 1
estramustine 61.1 3
cpsa 55.9 2
goserelin 55.4 1
genes like me logo Genes that share compounds with PROS1: view

Transcripts for PROS1 Gene

Unigene Clusters for PROS1 Gene

Protein S (alpha):
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for PROS1 Gene

No ASD Table

Relevant External Links for PROS1 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for PROS1 Gene

mRNA expression in normal human tissues for PROS1 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for PROS1 Gene

This gene is overexpressed in Liver (7.4) and Artery - Aorta (4.6).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for PROS1 Gene

SOURCE GeneReport for Unigene cluster for PROS1 Gene Hs.64016

mRNA Expression by UniProt/SwissProt for PROS1 Gene

Tissue specificity: Plasma
genes like me logo Genes that share expressions with PROS1: view

Orthologs for PROS1 Gene

This gene was present in the common ancestor of chordates.

Orthologs for PROS1 Gene

Organism Taxonomy Gene Similarity Type Details
(Pan troglodytes)
Mammalia PROS1 35
  • 99.65 (n)
  • 99.41 (a)
PROS1 36
  • 99 (a)
(Bos Taurus)
Mammalia PROS1 35
  • 87.6 (n)
  • 82.07 (a)
PROS1 36
  • 81 (a)
(Canis familiaris)
Mammalia PROS1 35
  • 81.05 (n)
  • 77.49 (a)
PROS1 36
  • 76 (a)
(Mus musculus)
Mammalia Pros1 35
  • 82.2 (n)
  • 79.82 (a)
Pros1 16
Pros1 36
  • 79 (a)
(Monodelphis domestica)
Mammalia PROS1 36
  • 68 (a)
(Ornithorhynchus anatinus)
Mammalia PROS1 36
  • 65 (a)
(Rattus norvegicus)
Mammalia Pros1 35
  • 83.43 (n)
  • 80.42 (a)
(Gallus gallus)
Aves PROS1 35
  • 70.44 (n)
  • 64.86 (a)
PROS1 36
  • 63 (a)
(Anolis carolinensis)
Reptilia PROS1 36
  • 56 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia pros1 35
  • 64.53 (n)
  • 61.31 (a)
(Danio rerio)
Actinopterygii pros1 35
  • 55.11 (n)
  • 53.99 (a)
pros1 36
  • 50 (a)
sea squirt
(Ciona savignyi)
Ascidiacea -- 36
  • 18 (a)
-- 36
  • 34 (a)
Species with no ortholog for PROS1:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • African malaria mosquito (Anopheles gambiae)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • baker's yeast (Saccharomyces cerevisiae)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • fruit fly (Drosophila melanogaster)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)
  • worm (Caenorhabditis elegans)

Evolution for PROS1 Gene

Gene Tree for PROS1 (if available)
Gene Tree for PROS1 (if available)

Paralogs for PROS1 Gene

Paralogs for PROS1 Gene Pseudogenes for PROS1 Gene

genes like me logo Genes that share paralogs with PROS1: view

Variants for PROS1 Gene

Sequence variations from dbSNP and Humsavar for PROS1 Gene

SNP ID Clin Chr 03 pos Sequence Context AA Info Type MAF
rs6121 -- 93,927,256(-) GACCC(G/T)GAAAC reference, synonymous-codon
rs6122 Thrombophilia due to protein S deficiency, autosomal dominant (THPH5) 93,927,251(-) GGAAA(C/T)GGTAA reference, missense
rs6123 -- 93,874,275(-) TGTCC(A/G)TCAGT reference, synonymous-codon
rs1130095 -- 93,973,718(-) GGCGC(C/T)GCTGG missense, reference
rs1130097 -- 93,900,869(-) CATCC(C/G/T)AGGAG reference, missense

Structural Variations from Database of Genomic Variants (DGV) for PROS1 Gene

Variant ID Type Subtype PubMed ID
nsv834764 CNV Loss 17160897
nsv877161 CNV Gain 21882294
esv2657355 CNV Deletion 23128226

Relevant External Links for PROS1 Gene

HapMap Linkage Disequilibrium report
Human Gene Mutation Database (HGMD)
Locus Specific Mutation Databases (LSDB)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for PROS1 Gene

Disorders for PROS1 Gene

(2) OMIM Diseases for PROS1 Gene (176880)


  • Thrombophilia due to protein S deficiency, autosomal dominant (THPH5) [MIM:612336]: A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. Based on the plasma levels of total and free PROS1 as well as the serine protease-activated protein C cofactor activity, three types of THPH5 have been described: type I, characterized by reduced total and free PROS1 levels together with reduced anticoagulant activity; type III, in which only free PROS1 antigen and PROS1 activity levels are reduced; and the rare type II which is characterized by normal concentrations of both total and free PROS1 antigen, but low cofactor activity. {ECO:0000269 PubMed:10447256, ECO:0000269 PubMed:10613647, ECO:0000269 PubMed:10706858, ECO:0000269 PubMed:10790208, ECO:0000269 PubMed:11372770, ECO:0000269 PubMed:11776305, ECO:0000269 PubMed:11858485, ECO:0000269 PubMed:11927129, ECO:0000269 PubMed:12351389, ECO:0000269 PubMed:12632031, ECO:0000269 PubMed:15238143, ECO:0000269 PubMed:15712227, ECO:0000269 PubMed:7482398, ECO:0000269 PubMed:7545463, ECO:0000269 PubMed:7579449, ECO:0000269 PubMed:7803790, ECO:0000269 PubMed:8298131, ECO:0000269 PubMed:8639833, ECO:0000269 PubMed:8701404, ECO:0000269 PubMed:8765219, ECO:0000269 PubMed:8781426, ECO:0000269 PubMed:8943854, ECO:0000269 PubMed:8977443, ECO:0000269 PubMed:9031443, ECO:0000269 PubMed:9241758, ECO:0000269 Ref.15}. Note=The disease is caused by mutations affecting the gene represented in this entry.
  • Thrombophilia due to protein S deficiency, autosomal recessive (THPH6) [MIM:614514]: A very rare and severe hematologic disorder resulting in thrombosis and secondary hemorrhage usually beginning in early infancy. Some affected individuals develop neonatal purpura fulminans, multifocal thrombosis, or intracranial hemorrhage. {ECO:0000269 PubMed:20484936}. Note=The disease is caused by mutations affecting the gene represented in this entry.

(24) Novoseek inferred disease relationships for PROS1 Gene

Disease -log(P) Hits PubMed IDs
protein s deficiency 88.9 33
prostate cancer 78.1 99
prostate carcinoma 45.8 5
chronic prostatitis 44.1 5
prostatitis 38.2 4

Genatlas disease for PROS1 Gene

thrombosis,recurrent (pro-S deficiency),types I with reduced level of antigen and activity,type II with normal antigen level,reduced activity but excluding most cases of type III

Relevant External Links for PROS1

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
genes like me logo Genes that share disorders with PROS1: view

Publications for PROS1 Gene

  1. Protein S gene analysis reveals the presence of a cosegregating mutation in most pedigrees with type I but not type III PS deficiency. (PMID: 10447256) Espinosa-Parrilla Y. … Sala N. (Hum. Mutat. 1999) 3 4 23
  2. Characterization and structural impact of five novel PROS1 mutations in eleven protein S-deficient families. (PMID: 11776305) Andersen B.D. … Thorsen S. (Thromb. Haemost. 2001) 3 4 23
  3. A large deletion of the PROS1 gene in a deep vein thrombosis patient with protein S deficiency. (PMID: 17938802) Yin T. … Miyata T. (Thromb. Haemost. 2007) 3 23 48
  4. PROS1 analysis in 87 pedigrees with hereditary protein S deficiency demonstrates striking genotype-phenotype associations. (PMID: 18435454) Ten Kate M.K. … van der Meer J. (Hum. Mutat. 2008) 3 23 48
  5. Prevalence of genetic mutations in protein S, protein C and antithrombin genes in Japanese patients with deep vein thrombosis. (PMID: 18954896) Miyata T. … Ikeda Y. (Thromb. Res. 2009) 3 23 48

Products for PROS1 Gene

Sources for PROS1 Gene

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