C4BPB Antibody

Background:

This gene encodes a member of a superfamily of proteinscomposed predominantly of tandemly arrayed short consensus repeatsof approximately 60 amino acids. A single, unique beta-chainencoded by this gene assembles with seven identical alpha-chainsinto the predominant isoform of C4b-binding protein, a multimericprotein that controls activation of the complement cascade throughthe classical pathway. C4b-binding protein has a regulatory role inthe coagulation system also, mediated through the beta-chainbinding of protein S, a vitamin K-dependent protein that serves asa cofactor of activated protein C. The genes encoding both alphaand beta chains are located adjacent to each other on humanchromosome 1 in the regulator of complement activation genecluster. Alternative splicing gives rise to multiple transcriptvariants.