C4BPB

complement component 4 binding protein beta

Summary

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]

Known Variants28 total

rsidPosition (GRCh37)AllelesClassClinVar
rs7515151791:207,262,905T/C—uncertain significance
rs1468461491:207,262,911C/T—likely benign
rs13726657251:207,262,932A/G—uncertain significance
rs127115131:207,262,980G/T—benign
rs111201841:207,263,539C/T—benign
rs1482052721:207,263,660C/T—benign
rs560736901:207,263,699C/T—benign
rs3696786651:207,263,736G/C—likely benign
rs7715738561:207,263,823C/T—uncertain significance
rs66900371:207,263,829A/G—benign
rs7543037211:207,265,067C/T—uncertain significance
rs1429096541:207,265,108C/T—likely benign
rs794434141:207,265,173C/A—likely benign
rs561140051:207,265,430G/A—benign
rs560543491:207,269,855C/T—benign
rs38139481:207,269,858T/Cintron variantbenign
rs89421:207,269,919C/T—benign
rs1444983321:207,271,511G/A—likely benign
rs7802725261:207,271,573C/T—likely benign
rs12643224371:207,271,576G/C—uncertain significance
rs1481160191:207,271,587A/C—uncertain significance
rs786676071:207,271,862T/G—benign
rs7685175051:207,273,174A/C—uncertain significance
rs16843971011:207,273,181T/G—uncertain significance
rs14199828481:207,273,206A/G—uncertain significance
rs1124578051:207,273,425G/A—benign
rs50080961:207,273,435G/A—benign
rs557049641:207,273,492G/A—benign

Gene information from NCBI Gene. Variant classifications from ClinVar.