Selected-GenAtlas references SOURCE GeneCards NCBI Gene Swiss-Prot Ensembl
HGNC UniGene Nucleotide OMIM UCSC
Home Page
FLASH GENE
Symbol C4BPA contributors: mct - updated : 24-12-2013
HGNC name complement component 4 binding protein, alpha
HGNC id 1325
Location 1q32.1      Physical location : 207.277.606 - 207.318.308
Synonym name
  • proline-rich protein
  • C4b binding protein, alpha chain
    • Synonym symbol(s) PRP, C4BP
    DNA
    TYPE functioning gene
    STRUCTURE 40.73 kb     12 Exon(s)
    Genomic sequence alignment details
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked Y status confirmed
    Map see RCA@
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    12 - 2256 - 597 - -
  • a 48 aa signal peptide of 5.4 kda
  • a 549 aa mature peptide of 61.7 kda
    		•
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveliver   predominantly
    Reproductivefemale systemuteruscervix moderately
    Respiratorylung   moderately
    Urinarybladder   highly
    cell lineage
    cell lines
    fluid/secretion plasma
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • eight short consensus repeats, sushi structure (see F13B, SRCP1) region common to Ba region in factor B
  • four complement control protein domains
    • conjugated GlycoP
    HOMOLOGY
    interspecies homolog to rattus C4bpa (59.6 pc)
    Homologene
    FAMILY
    CATEGORY immunity/defense , regulatory
    SUBCELLULAR LOCALIZATION extracellular
        plasma membrane
        intracellular
    intracellular,cytoplasm
    intracellular,nucleus
    basic FUNCTION
  • playing a role of regulator of C3 convertase of classical pathway
  • facilitating the factor I-mediated proteolytic cleavage of the active forms of complement effectors CR1/C4B into their inactive forms
  • controlling the classical pathway of complement activation
  • hydrolyzing the complement fragment C4b
  • accelerating the degradation of the C4bC2a complex (C3 convertase) by dissociating the complement fragment C2a
  • soluble, multiple-subunit inhibitor of complement that circulates in blood
  • is recruited to the S. aureus surface where it functions to inhibit C4 complement effectors
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS immunity/defense
    text
  • innate immune response
  • complement activation, classical pathway
    • PATHWAY
    metabolism
    signaling
    a component
  • complexing with C4BPB
    • INTERACTION
    DNA multivalent interaction with DNA or surface bound glycosaminoglycans
    RNA
    small molecule
    protein
  • C4, alpha chain
  • binding as a cofactor to C3b/C4b inactivator (C3bINA)
  • interacting with anticoagulant protein S and with serum amyloid P component
  • PTX3 binds to the classical and lectin pathway regulator C4b-binding protein (C4BPA)
  • PRELP interact with the complement inhibitor C4B-binding protein, C4BPB, and C4BPA
  • major inhibitor of the classical complement and lectin pathway
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Susceptibility
  • to idiopathic, recurrent, spontaneous pregnancy loss
    • Variant & Polymorphism other
  • R120H, I126T, and the G423T mutations affected the expression level and/or the ability of recombinant C4BP to serve as cofactor for factor I, and are associated with idiopathic, recurrent, spontaneous pregnancy loss
    • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS