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protein S; vitamin K-dependent protein S (PROS1, PROS)

Function: - anticoagulant plasma protein - cofactor to activated protein C in the degradation of coagulation factor Va & factor VIIIa - helps to prevent coagulation - stimulates fibrinolysis - vitamin K-dependent binding protein - cofactor for protein C - binds C4b-binding protein; 50% is protein bound - only free form of protein S has biological activity Structure: - contains 4 EGF-like domains - the iron & 2-oxoglutarate dependent 3-hydroxylation of Asp & Asn is (R) stereospecific within EGF domains - contains 1 Gla (gamma-carboxy-glutamate) domain - contains 2 laminin G-like domains Compartment: secreted, plasma Expression: - presumablu expressed in liver & secreted into plasma - plasma activity may be reduced as a result of acute thrombosis - anticoagulation may lower activity of protein S in plasma Pathology: - defects in PROS1 are the cause of protein S deficiency - individuals with heterozygous protein S deficiency often develop thromboembolic disease before age 30

Interactions

molecular events

Related

coagulation cascade protein S activity in plasma (protein S assay) protein S deficiency

Specific

protein-S alpha protein-S beta

General

coagulation factor endogenous anticoagulant

Properties

SIZE: entity length = 676 aa MW = 75 kD COMPARTMENT: plasma MOTIF: signal sequence {1-24} Gla {42-87} MOTIF: cysteine residue {C58} MODIFICATION: cysteine residue {C63} cysteine residue {C63} MODIFICATION: cysteine residue {C58} Thrombin-sensitive {88-116} EGF domain {117-155} MOTIF: cysteine residue {C121} MODIFICATION: cysteine residue {C134} cysteine residue {C126} MODIFICATION: cysteine residue {C143} cysteine residue {C134} MODIFICATION: cysteine residue {C121} cysteine residue {C143} MODIFICATION: cysteine residue {C126} cysteine residue {C145} MODIFICATION: cysteine residue {C154} cysteine residue {C154} MODIFICATION: cysteine residue {C145} EGF domain {157-200} MOTIF: cysteine residue {C161} MODIFICATION: cysteine residue {C175} cysteine residue {C171} MODIFICATION: cysteine residue {C184} cysteine residue {C175} MODIFICATION: cysteine residue {C161} cysteine residue {C184} MODIFICATION: cysteine residue {C171} cysteine residue {C186} MODIFICATION: cysteine residue {C199} cysteine residue {C199} MODIFICATION: cysteine residue {C186} EGF domain {201-242} MOTIF: cysteine residue {C205} MODIFICATION: cysteine residue {C217} cysteine residue {C212} MODIFICATION: cysteine residue {C226} cysteine residue {C217} MODIFICATION: cysteine residue {C205} cysteine residue {C226} MODIFICATION: cysteine residue {C212} cysteine residue {C228} MODIFICATION: cysteine residue {C241} cysteine residue {C241} MODIFICATION: cysteine residue {C228} EGF domain {243-283} MOTIF: cysteine residue {C247} MODIFICATION: cysteine residue {C256} cysteine residue {C252} MODIFICATION: cysteine residue {C265} cysteine residue {C256} MODIFICATION: cysteine residue {C247} cysteine residue {C265} MODIFICATION: cysteine residue {C252} cysteine residue {C267} MODIFICATION: cysteine residue {C282} cysteine residue {C282} MODIFICATION: cysteine residue {C267} Laminin G-like 1 {299-475} MOTIF: cysteine residue {C449} MODIFICATION: cysteine residue {C475} cysteine residue {C475} MODIFICATION: cysteine residue {C449} Laminin G-like 2 {484-666} MOTIF: asparagine residue {499} N-glycosylation site {N499} N-glycosylation site {N509} N-glycosylation site {N530} cysteine residue {C639} MODIFICATION: cysteine residue {C666} cysteine residue {C666} MODIFICATION: cysteine residue {C639} SECRETED-BY: hepatocyte megakaryocyte

Database Correlations

OMIM correlations MORBIDMAP 176880 UniProt P07225 PFAM correlations Entrez Gene 5627 Kegg hsa:5627

References

  1. UniProt :accession P07225
  2. GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/PROS1
  3. SeattleSNPs http://pga.gs.washington.edu/data/pros1/
  4. Medical Knowledge Self Assessment Program (MKSAP) 11, 16. American College of Physicians, Philadelphia 1998, 2012

Component-of

factor ix/factor vii/factor x/protein c/protein s/prothrombin/prothrombin complex concentrate prothrombin complex concentrate (Autoplex-T, Kcentra)