Aliases for C4BPA Gene
External Ids for C4BPA Gene
Previous HGNC Symbols for C4BPA Gene
Previous GeneCards Identifiers for C4BPA Gene
This gene encodes a member of a superfamily of proteins composed predominantly of tandemly arrayed short consensus repeats of approximately 60 amino acids. Along with a single, unique beta-chain, seven identical alpha-chains encoded by this gene assemble into the predominant isoform of C4b-binding protein, a multimeric protein that controls activation of the complement cascade through the classical pathway. 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. Two pseudogenes of this gene are also found in the cluster. [provided by RefSeq, Jul 2008]
GeneCards Summary for C4BPA Gene
C4BPA (Complement Component 4 Binding Protein Alpha) is a Protein Coding gene. Diseases associated with C4BPA include Protein S Deficiency and Acute Poststreptococcal Glomerulonephritis. Among its related pathways are Immune response Lectin induced complement pathway and Pertussis. An important paralog of this gene is CSMD3.
UniProtKB/Swiss-Prot Summary for C4BPA Gene
Controls the classical pathway of complement activation. It binds as a cofactor to C3b/C4b inactivator (C3bINA), which then hydrolyzes the complement fragment C4b. It also accelerates the degradation of the C4bC2a complex (C3 convertase) by dissociating the complement fragment C2a. Alpha chain binds C4b. It interacts also with anticoagulant protein S and with serum amyloid P component.