InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PROS1

Summary

InnateDB Protein

IDBP-242005.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

PROS1

Protein Name

protein S (alpha)

Synonyms

PROS; PS21; PS22; PS23; PS24; PS25; PSA; THPH5; THPH6;

Species

Homo sapiens

Ensembl Protein

ENSP00000377783

InnateDB Gene

IDBG-46049 (PROS1)

Protein Structure

UniProt Annotation

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.

Subcellular Localization

Secreted.

Disease Associations

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:0000269PubMed:10447256, ECO:0000269PubMed:10613647, ECO:0000269PubMed:10706858, ECO:0000269PubMed:10790208, ECO:0000269PubMed:11372770, ECO:0000269PubMed:11776305, ECO:0000269PubMed:11858485, ECO:0000269PubMed:11927129, ECO:0000269PubMed:12351389, ECO:0000269PubMed:12632031, ECO:0000269PubMed:15238143, ECO:0000269PubMed:15712227, ECO:0000269PubMed:7482398, ECO:0000269PubMed:7545463, ECO:0000269PubMed:7579449, ECO:0000269PubMed:7803790, ECO:0000269PubMed:8298131, ECO:0000269PubMed:8639833, ECO:0000269PubMed:8701404, ECO:0000269PubMed:8765219, ECO:0000269PubMed:8781426, ECO:0000269PubMed:8943854, ECO:0000269PubMed:8977443, ECO:0000269PubMed:9031443, ECO:0000269PubMed:9241758, ECO:0000269Ref.14}. 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:0000269PubMed:20484936}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Plasma.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 9 experimentally validated interaction(s) in this database.

Experimentally validated
Total	9 [view]
Protein-Protein	9 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004866	endopeptidase inhibitor activity
GO:0005509	calcium ion binding
GO:0005515	protein binding

Biological Process

GO:0002576	platelet degranulation
GO:0006508	proteolysis
GO:0007596	blood coagulation
GO:0010951	negative regulation of endopeptidase activity
GO:0017187	peptidyl-glutamic acid carboxylation
GO:0030168	platelet activation
GO:0030449	regulation of complement activation
GO:0042730	fibrinolysis
GO:0043687	post-translational protein modification
GO:0044267	cellular protein metabolic process
GO:0045087	innate immune response (InnateDB)
GO:0050900	leukocyte migration

Cellular Component

GO:0000139	Golgi membrane
GO:0005576	extracellular region
GO:0005615	extracellular space
GO:0005789	endoplasmic reticulum membrane
GO:0005796	Golgi lumen
GO:0031093	platelet alpha granule lumen
GO:0072562	blood microparticle

Protein Structure and Domains

PDB ID

InterPro

IPR000294 Gamma-carboxyglutamic acid-rich (GLA) domain
IPR000742 Epidermal growth factor-like domain
IPR001791 Laminin G domain
IPR001881 EGF-like calcium-binding domain
IPR008985 Concanavalin A-like lectin/glucanases superfamily

PFAM

PF00594
PF00008
PF00054
PF02210
PF07645

PRINTS

PR00001

PIRSF

SMART

SM00069
SM00181
SM00210
SM00282
SM00179

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt