C4BPB - complement component 4 binding protein beta Gene

中文名称：补体成分 4 结合蛋白 β

种属: Homo sapiens

同用名: C4BP

关于 C4BPB

功能概要

该基因编码的蛋白质超家族成员主要由大约 60 个氨基酸的串联排列的短共有重复序列组成。由该基因编码的单一、独特的 β 链与七个相同的 α 链组装成 C4b 结合蛋白的主要亚型，C4b 结合蛋白是一种通过经典途径控制补体级联激活的多聚体蛋白。 C4b 结合蛋白在凝血系统中也具有调节作用，通过蛋白 S 的 β 链结合介导，蛋白 S 是一种维生素 K 依赖性蛋白，可作为活化蛋白 C 的辅助因子。编码 α 和 β 链的基因是位于人类 1 号染色体上相邻的补体激活基因簇调节因子。选择性剪接会产生多种转录本变体。[RefSeq 提供，2008 年 7 月]

This gene encodes 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. [provided by RefSeq, Jul 2008]