C4BPB is a member of a superfamily of proteins composed predominantly of tandemly arrayed short consensus repeats of approximately 60 amino acids. A single, unique beta-chain encoded by this gene assembles with seven identical alpha-chains into the predominant isoform of C4b-binding protein, a multimeric protein that controls activation of the complement cascade through the classical pathway. C4b-binding protein has a regulatory role in the coagulation system also, mediated through the beta-chain binding of protein S, a vitamin K-dependent protein that serves as a cofactor of activated protein C. 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. Alternative splicing gives rise to multiple transcript variants
Synonyms: C4b binding protein beta chain, C4b-binding protein beta chain, C4BP, C4BPB, C4BPB_HUMAN, Complement component 4 binding protein beta, Complement component 4 binding protein beta chain, Complement component 4-binding protein beta, OTTHUMP00000034288, OTTHUMP00000034289, OTTHUMP00000034375, OTTHUMP0000003474, Proline rich protein, PRP.