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/Cuncertain significance
rs1468461491:207,262,911C/Tlikely benign
rs13726657251:207,262,932A/Guncertain significance
rs127115131:207,262,980G/Tbenign
rs111201841:207,263,539C/Tbenign
rs1482052721:207,263,660C/Tbenign
rs560736901:207,263,699C/Tbenign
rs3696786651:207,263,736G/Clikely benign
rs7715738561:207,263,823C/Tuncertain significance
rs66900371:207,263,829A/Gbenign
rs7543037211:207,265,067C/Tuncertain significance
rs1429096541:207,265,108C/Tlikely benign
rs794434141:207,265,173C/Alikely benign
rs561140051:207,265,430G/Abenign
rs560543491:207,269,855C/Tbenign
rs38139481:207,269,858T/Cintron variantbenign
rs89421:207,269,919C/Tbenign
rs1444983321:207,271,511G/Alikely benign
rs7802725261:207,271,573C/Tlikely benign
rs12643224371:207,271,576G/Cuncertain significance
rs1481160191:207,271,587A/Cuncertain significance
rs786676071:207,271,862T/Gbenign
rs7685175051:207,273,174A/Cuncertain significance
rs16843971011:207,273,181T/Guncertain significance
rs14199828481:207,273,206A/Guncertain significance
rs1124578051:207,273,425G/Abenign
rs50080961:207,273,435G/Abenign
rs557049641:207,273,492G/Abenign

Gene information from NCBI Gene. Variant classifications from ClinVar.