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Protein Page:
PROS1 (human)

Overview
PROS1 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. Defects in PROS1 are the cause of thrombophilia due to protein S deficiency, autosomal dominant (THPH5). A hemostatic disorder characterized by impaired regulation of blood coagulation and a tendency to recurrent venous thrombosis. However, many adults with heterozygous disease may be asymptomatic. Based on the plasma levels of total and free PROS1 antigen as well as the serine protease-activated protein C cofactor activity, three types of PROS1D have been described: type I, characterized by reduced total and free PROS1 antigen 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. Defects in PROS1 are the cause of thrombophilia due to protein S deficiency, autosomal recessive (THPH6). 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. Note: This description may include information from UniProtKB.
Protein type: Secreted; Secreted, signal peptide
Cellular Component: Golgi membrane; extracellular space; endoplasmic reticulum membrane; Golgi lumen; extracellular region
Molecular Function: endopeptidase inhibitor activity; calcium ion binding
Biological Process: platelet activation; fibrinolysis; platelet degranulation; cellular protein metabolic process; regulation of complement activation; innate immune response; proteolysis; blood coagulation; post-translational protein modification; leukocyte migration; peptidyl-glutamic acid carboxylation
Reference #:  P07225 (UniProtKB)
Alt. Names/Synonyms: PROS; PROS1; protein S (alpha); protein Sa; PS21; PS22; PS23; PS24; PS25; PSA; vitamin K-dependent plasma protein S; Vitamin K-dependent protein S
Gene Symbols: PROS1
Molecular weight: 75,123 Da
Basal Isoelectric point: 5.48  Predict pI for various phosphorylation states
Select Structure to View Below

PROS1

Protein Structure Not Found.


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Modification Sites and Domains  

Modification Sites in Parent Protein, Orthologs, and Isoforms  
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 T49 ANSLLEETKQGNLER
1 1 T78-p VFENDPEtDYFYPKY
0 1 T104-p FTAARQStNAYPDLR
0 1 S122-p NAIPDQCsPLPCNED
0 1 Y259-p CAQLCVNyPGGYTCy
0 1 Y266-p yPGGYTCyCDGKKGF
  mouse

 
T49-p ANTLFEEtMKGNLER
T78 VFENNPETDYFYPKY
A104 FHAARQSANAYPDLR
D122 KAISDQCDPIPCNED
F259 CAQLCVNFPGGYSCY
Y266 FPGGYSCYCDGKKGF
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