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C4BPA is 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.
Synonyms: C4b-binding protein alpha chain, C4bp, C4bpa, C4BPA_HUMAN, Complement component 4 binding protein, Proline-rich protein, PrP, RP11-164O23.4.