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Vitamin K–Dependent Protein S : Beyond the Protein C Pathway

Dahlbäck, Björn LU (2017) In Seminars in Thrombosis and Hemostasis
Abstract

Protein S is a vitamin K–dependent plasma glycoprotein circulating in plasma at a concentration of around 350 nM. Approximately 60% of protein S in human plasma is bound to the complement regulatory protein C4b-binding protein (C4BP) in a high-affinity, high-molecular-weight complex. Protein S in plasma has multiple anticoagulant properties and heterozygous protein S deficiency is associated with increased risk of venous thrombosis. Homozygous deficiency in man and mice is associated with severe thrombosis in fetal life, defects in the vascular system development, and not compatible with life. Protein S has additional functions beyond being an anticoagulant. It affects the complement regulatory properties of C4BP, and moreover, protein... (More)

Protein S is a vitamin K–dependent plasma glycoprotein circulating in plasma at a concentration of around 350 nM. Approximately 60% of protein S in human plasma is bound to the complement regulatory protein C4b-binding protein (C4BP) in a high-affinity, high-molecular-weight complex. Protein S in plasma has multiple anticoagulant properties and heterozygous protein S deficiency is associated with increased risk of venous thrombosis. Homozygous deficiency in man and mice is associated with severe thrombosis in fetal life, defects in the vascular system development, and not compatible with life. Protein S has additional functions beyond being an anticoagulant. It affects the complement regulatory properties of C4BP, and moreover, protein S interacts with tyrosine kinase receptors of the TAM family, which comprises Tyro3, Axl, and Mer. The TAM receptor interaction is important for the ability of protein S to stimulate phagocytosis of apoptotic cells. This review will discuss the multiple functions of protein S, describing its role as cofactor to activated protein C with a subsequent focus on the other functions of protein S.

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author
organization
publishing date
type
Contribution to journal
publication status
epub
subject
keywords
Axl, blood coagulation, C4b-binding protein, C4BP, Gas6, Mer, protein C, Tyro3
in
Seminars in Thrombosis and Hemostasis
publisher
Georg Thieme Verlag
external identifiers
  • scopus:85029749373
  • pmid:28905350
ISSN
0094-6176
DOI
10.1055/s-0037-1604092
language
English
LU publication?
yes
id
0d43601b-e1d1-4e8c-9a8a-8e5fa10f2334
date added to LUP
2017-10-10 15:32:29
date last changed
2018-03-11 04:44:09
@article{0d43601b-e1d1-4e8c-9a8a-8e5fa10f2334,
  abstract     = {<p>Protein S is a vitamin K–dependent plasma glycoprotein circulating in plasma at a concentration of around 350 nM. Approximately 60% of protein S in human plasma is bound to the complement regulatory protein C4b-binding protein (C4BP) in a high-affinity, high-molecular-weight complex. Protein S in plasma has multiple anticoagulant properties and heterozygous protein S deficiency is associated with increased risk of venous thrombosis. Homozygous deficiency in man and mice is associated with severe thrombosis in fetal life, defects in the vascular system development, and not compatible with life. Protein S has additional functions beyond being an anticoagulant. It affects the complement regulatory properties of C4BP, and moreover, protein S interacts with tyrosine kinase receptors of the TAM family, which comprises Tyro3, Axl, and Mer. The TAM receptor interaction is important for the ability of protein S to stimulate phagocytosis of apoptotic cells. This review will discuss the multiple functions of protein S, describing its role as cofactor to activated protein C with a subsequent focus on the other functions of protein S.</p>},
  author       = {Dahlbäck, Björn},
  issn         = {0094-6176},
  keyword      = {Axl,blood coagulation,C4b-binding protein,C4BP,Gas6,Mer,protein C,Tyro3},
  language     = {eng},
  publisher    = {Georg Thieme Verlag},
  series       = {Seminars in Thrombosis and Hemostasis},
  title        = {Vitamin K–Dependent Protein S : Beyond the Protein C Pathway},
  url          = {http://dx.doi.org/10.1055/s-0037-1604092},
  year         = {2017},
}