Gene Symbol

C4BPA

Aliases

C4BP, PRP

Entrez Gene ID

722

Gene Name

Complement component 4 binding protein alpha

Chromosomal Location

1q32.2

HGNC ID

HGNC:1325

Summary

This gene encodes a member of a superfamily of proteins composed predominantly of tandemly arrayed short consensus repeats of approximately 60 amino acids. Along with a single, unique beta-chain, seven identical alpha-chains encoded by this gene assemble into the predominant isoform of C4b-binding protein, a multimeric protein that controls activation of the complement cascade through the classical pathway. The genes encoding both alpha and beta chains are located adjacent to each other on human chromosome 1 in the regulator of complement activation gene cluster. Two pseudogenes of this gene are also found in the cluster. [provided by RefSeq, Jul 2008]

Function

Controls the classical pathway of complement activation. It binds as a cofactor to C3b/C4b inactivator (C3bINA), which then hydrolyzes the complement fragment C4b. It also accelerates the degradation of the C4bC2a complex (C3 convertase) by dissociating the complement fragment C2a. Alpha chain binds C4b. It interacts also with anticoagulant protein S and with serum amyloid P component

P04003

PDB

2A55, 4B0F, 5HYP, 5HYT, 5HYU, 5HZP, 5I0Q

STRING	MINT	IntAct
ENSP00000356037		P04003