C4b-binding protein
C4b-binding protein (C4BP) is a protein complex involved in the complement system 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.
complement component 4 binding protein, alpha | |||||||
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Identifiers | |||||||
Symbol | C4BPA | ||||||
Alt. symbols | C4BP | ||||||
NCBI gene | 722 | ||||||
HGNC | 1325 | ||||||
OMIM | 120830 | ||||||
PDB | 4B0F | ||||||
RefSeq | NM_000715 | ||||||
UniProt | P04003 | ||||||
Other data | |||||||
Locus | Chr. 1 q32 | ||||||
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complement component 4 binding protein, beta | |||||||
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Identifiers | |||||||
Symbol | C4BPB | ||||||
Alt. symbols | C4BP | ||||||
NCBI gene | 725 | ||||||
HGNC | 1328 | ||||||
OMIM | 120831 | ||||||
RefSeq | NM_000716 | ||||||
UniProt | P20851 | ||||||
Other data | |||||||
Locus | Chr. 1 q32 | ||||||
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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.
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