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C4b-Binding Protein: A Forgotten Factor in Thrombosis and Hemostasis

Dahlbäck, Björn LU (2011) In Seminars in Thrombosis and Hemostasis 37(4). p.355-361
Abstract
Blood coagulation and complement pathways are two important natural defense systems. The high affinity interaction between the anticoagulant vitamin K-dependent protein S and the complement regulator C4b-binding protein (C4BP) is a direct physical link between the two systems. In human plasma, similar to 70% of total protein S circulates in complex with C4BP; the remaining is free. The anticoagulant activity of protein S is mainly expressed by the free form, although the protein S-C4BP complex has recently been shown to have some anticoagulant activity. The high affinity, binding of protein S to C4BP provides C4BP with the ability to bind to negatively charged phospholipid membranes, which serves the purpose of localizing complement... (More)
Blood coagulation and complement pathways are two important natural defense systems. The high affinity interaction between the anticoagulant vitamin K-dependent protein S and the complement regulator C4b-binding protein (C4BP) is a direct physical link between the two systems. In human plasma, similar to 70% of total protein S circulates in complex with C4BP; the remaining is free. The anticoagulant activity of protein S is mainly expressed by the free form, although the protein S-C4BP complex has recently been shown to have some anticoagulant activity. The high affinity, binding of protein S to C4BP provides C4BP with the ability to bind to negatively charged phospholipid membranes, which serves the purpose of localizing complement regulatory activity close to the membrane. Even though C4BP does not directly affect the coagulation system, it still influences the regulation of blood coagulation through its interaction with protein S. This is particularly important in states of inherited deficiency of protein S where the tight binding of protein S to C4BP results in a pronounced and selective drop in concentration of free protein S, whereas the concentration of protein S in complex with C4BP remains relatively unchanged. This review summarizes the current knowledge on C4BP with respect to its association with thrombosis and hemostasis. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
C4b-binding protein, protein S, protein C, complement, coagulation
in
Seminars in Thrombosis and Hemostasis
volume
37
issue
4
pages
355 - 361
publisher
Georg Thieme Verlag
external identifiers
  • wos:000292278800004
  • scopus:79961053729
ISSN
1098-9064
DOI
10.1055/s-0031-1276584
language
English
LU publication?
yes
id
d2e17bd2-9297-4bc0-8d71-e88945824137 (old id 2029170)
date added to LUP
2011-08-02 08:57:39
date last changed
2017-10-01 04:11:31
@article{d2e17bd2-9297-4bc0-8d71-e88945824137,
  abstract     = {Blood coagulation and complement pathways are two important natural defense systems. The high affinity interaction between the anticoagulant vitamin K-dependent protein S and the complement regulator C4b-binding protein (C4BP) is a direct physical link between the two systems. In human plasma, similar to 70% of total protein S circulates in complex with C4BP; the remaining is free. The anticoagulant activity of protein S is mainly expressed by the free form, although the protein S-C4BP complex has recently been shown to have some anticoagulant activity. The high affinity, binding of protein S to C4BP provides C4BP with the ability to bind to negatively charged phospholipid membranes, which serves the purpose of localizing complement regulatory activity close to the membrane. Even though C4BP does not directly affect the coagulation system, it still influences the regulation of blood coagulation through its interaction with protein S. This is particularly important in states of inherited deficiency of protein S where the tight binding of protein S to C4BP results in a pronounced and selective drop in concentration of free protein S, whereas the concentration of protein S in complex with C4BP remains relatively unchanged. This review summarizes the current knowledge on C4BP with respect to its association with thrombosis and hemostasis.},
  author       = {Dahlbäck, Björn},
  issn         = {1098-9064},
  keyword      = {C4b-binding protein,protein S,protein C,complement,coagulation},
  language     = {eng},
  number       = {4},
  pages        = {355--361},
  publisher    = {Georg Thieme Verlag},
  series       = {Seminars in Thrombosis and Hemostasis},
  title        = {C4b-Binding Protein: A Forgotten Factor in Thrombosis and Hemostasis},
  url          = {http://dx.doi.org/10.1055/s-0031-1276584},
  volume       = {37},
  year         = {2011},
}