C4b-binding protein (C4BP) is a

protein complex A protein complex or multiprotein complex is a group of two or more associated polypeptide chains. Protein complexes are distinct from multienzyme complexes, in which multiple catalytic domains are found in a single polypeptide chain. Protein c ...

involved in the

complement system The complement system, also known as complement cascade, is a part of the immune system that enhances (complements) the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promote inflammation, and at ...

where it acts as inhibitor. C4BP has an octopus-like structure with a central stalk and seven branching alpha-chains. The main form of C4BP in human blood is composed of 7 identical alpha-chains and one unique beta-chain, which in turn binds anticoagulant, vitamin K-dependent

protein S Protein S (also known as PROS) is a vitamin K-dependent plasma glycoprotein synthesized in the liver. In the circulation, Protein S exists in two forms: a free form and a complex form bound to complement protein C4b-binding protein (C4BP). In huma ...

. C4BP is a large glycoprotein (500 kDa) with an estimated plasma concentration of 200 micrograms/mL synthesized mainly in the liver. The genes coding for C4BP α-chain (C4BPA) and β-chain (C4BPB) are located in the regulators of complement activation (RCA) gene cluster on the long arm of chromosome 1 in the vicinity of other complement inhibitors.

Functions

It inhibits the action the classical and the lectin pathways, more specifically C4. It also has ability to bind C3b. C4BP accelerates decay of

C3-convertase C3 convertase (''C4bC2b'', formerly ''C4b2a'') belongs to family of serine proteases and is necessary in innate immunity as a part of the complement system which eventuate in opsonisation of particles, release of inflammatory peptides, C5 conve ...

and is a cofactor for serine protease factor I which cleaves C4b and C3b. C4BP binds apoptotic and necrotic cells as well as DNA, to clean up after injury. The interaction with apoptotic and necrotic cells is mediated by the

Gla domain Vitamin K-dependent carboxylation/gamma-carboxyglutamic (GLA) domain is a protein domain that contains post-translational modifications of many glutamate residues by vitamin K-dependent carboxylation to form γ-carboxyglutamate (Gla). Proteins wit ...

of protein S and does not affect the ability of C4BP to inhibit complement. A number of bacterial and fungal pathogens capture human C4BP and use it to prevent binding of C4b, which allows them to establish infection. No full deficiency of C4BP has been found yet. C4BP interacts also with

heparin Heparin, also known as unfractionated heparin (UFH), is a medication and naturally occurring glycosaminoglycan. Since heparins depend on the activity of antithrombin, they are considered anticoagulants. Specifically it is also used in the treatm ...

C-reactive protein C-reactive protein (CRP) is an annular (ring-shaped) pentameric protein found in blood plasma, whose circulating concentrations rise in response to inflammation. It is an acute-phase protein of hepatic origin that increases following interleukin-6 ...

(CRP),

serum amyloid P component The serum amyloid P component (SAP) is the identical serum form of amyloid P component (AP), a 25kDa pentameric protein first identified as the pentagonal constituent of in vivo pathological deposits called "amyloid". APCS is its human gene. In ...

(SAP),

fibromodulin Fibromodulin is a protein that in humans is encoded by the ''FMOD'' gene. Fibromodulin is a 42kDa protein of a family of small interstitial leucine-rich repeat proteoglycans (SLRPs). It can have up to four N-linked keratan sulfate chains attached ...

osteoadherin Osteomodulin (also called osteoadherin or osteoadherin proteoglycan) is a protein that in humans is encoded by the ''OMD'' gene In biology, the word gene (from , ; "... Wilhelm Johannsen coined the word gene to describe the Mendelian units ...

, chondroadherin, proline arginine-rich end leucine-rich repeat protein (

PRELP Prolargin is a protein that in humans is encoded by the ''PRELP'' gene. The protein encoded by this gene is a leucine-rich repeat protein present in connective tissue extracellular matrix. This protein functions as a molecule anchoring basemen ...

), streptococcal M-proteins, gonococcal porins, Outer membrane protein A from

E. coli ''Escherichia coli'' (),Wells, J. C. (2000) Longman Pronunciation Dictionary. Harlow ngland Pearson Education Ltd. also known as ''E. coli'' (), is a Gram-negative, facultative anaerobic, rod-shaped, coliform bacterium of the genus ''Escher ...

, Ubiquitous surface protein 1 and 2 from

Moraxella ''Moraxella'' is a genus of gram-negative bacteria in the family Moraxellaceae. It is named after the Swiss ophthalmologist Victor Morax. The organisms are short rods, coccobacilli, or as in the case of ''Moraxella catarrhalis'', diplococci in ...

References

External links

* {{Complement system