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Official Full Name: complement component 4 binding protein beta
Also known as: C4BP
This gene encodes a member of a protein superfamily consisting mainly of short consensus repeats arranged in tandem of approximately 60 amino acids. A unique beta chain encoded by this gene assembles with seven identical alpha chains to form the major isoform of C4b-binding protein, a multimeric protein that controls the activation of the complement cascade through the classical pathway. The C4B-binding protein also has a regulatory role in the coagulation system, mediated through the binding of the beta chain of protein S, a vitamin K-dependent protein, that acts as a cofactor for the activation of protein C. On human chromosome 1, the genes encoding the α and β chains are adjacent to each other in the regulation of the complement activation gene cluster. Alternative splicing produces multiple transcript variants.