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FLASH GENE
Symbol C4BPA contributors: mct - updated : 24-12-2013
HGNC name complement component 4 binding protein, alpha
HGNC id 1325
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