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FLASH GENE
Symbol C5 contributors: mct/npt - updated : 02-05-2013
HGNC name complement component 5
HGNC id 1331
Corresponding disease
C5 complement component 5 deficiency
Location 9q33.2      Physical location : 123.714.615 - 123.812.554
Synonym name
  • anaphylatoxin C5a analog
  • C3 and PZP-like alpha-2-macroglobulin domain-containing
  • protein 4
    Synonym symbol(s) CPAMD4, MGC142298, FLJ17816, FLJ17822
    DNA
    TYPE functioning gene
    STRUCTURE 97.94 kb     41 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    Physical map
    CDK5RAP2 9q33.3 CDK5 regulatory subunit associated protein 2 LOC389788 9 similar to EGFL5 LOC392387 9 similar to Ahcy protein FBXW2 9q34 F-box and WD-40 domain protein 2 PSMD5 9q33.3 proteasome (prosome, macropain) 26S subunit, non-ATPase, 5 PHF19 9q34.11 PHD finger protein 19 TRAF1 9q33-q34 TNF receptor-associated factor 1 C5 9q33 complement component 5 CEP1 9q33 centrosomal protein 1 RAB14 9q32-q34.11 RAB14, member RAS oncogene family LOC392388 9 similar to vacuolar H+-ATPase B subunit GSN 9q33.3 gelsolin (amyloidosis, Finnish type) MOST2 9q34.11 MOST2 protein STOM 9q34.1 stomatin a1/3GTP 9q34.11 alpha-1,3-galactosyltransferase pseudogene DAB2IP 9q33.1-q33.3 DAB2 interacting protein LOC158135 9q34.11 similar to H23L24.3.p NDUFA8 9q32.4-q34.41 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8, 19kDa FLJ46909 9q34.11 FLJ46909 protein LHX6 9q34.11 LIM homeobox 6 MGC2734 MRRF 9q33-q34.1 mitochondrial ribosome recycling factor PTGS1 9q32-q33.3 prostaglandin-endoperoxide synthase 1 (prostaglandin G/H synthase and cyclooxygenase) LOC347168 9q34.11 similar to Olfactory receptor 1J1 OR1J5 9q34.11 olfactory receptor, family 1, subfamily J, member 5
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    41 - 5480 - 1676 - 2003 12794141
    EXPRESSION
    Type
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestiveliver    
    Lymphoid/Immunetonsils    
    Nervousbraindiencephalonhypothalamus  
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / hematopoieticbone marrow   
    cell lineage
    cell lines
    fluid/secretion plasma
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • one anaphylatoxin-like domain
  • one NTR domain
  • secondary structure an alpha 2
    conjugated GlycoP
    isoforms Precursor
    HOMOLOGY
    interspecies ortholog to murine Hc
    ortholog to rattus c5
    Homologene
    FAMILY
    CATEGORY immunity/defense
    SUBCELLULAR LOCALIZATION extracellular
        plasma membrane
        intracellular
    intracellular,cytoplasm
    basic FUNCTION
  • complement component 5, initiating, after activation, the assembly of the late complement components C5-C9 into the membrane attack complex
  • activation of the complement cascade and formation of C5a may play a role in the onset of acute coronary events by induction of MMPs in atherosclerotic lesions (
  • its activation elicits proteolytic cascades which eventually results in the cleavage of the C5 protein into two fragments, C5a and C5b
  • CELLULAR PROCESS cell communication
    PHYSIOLOGICAL PROCESS immunity/defense , inflammation
    text chemotaxis C5a
    PATHWAY
    metabolism
    signaling signal transduction
    a component
  • component 5 associating with the late components to form the membrane attack complex
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • C5b, the C345C domain of C5 and the FIMs of C7, which mediates reversible binding of C5 to C7 in solution, plays an essential role in MAC (membrane attack complex) formation and complement lytic activity
  • thrombin and C5 convertase are invariant partners, enhancing the terminal pathway via the generation of newly uncovered C5 intermediates
  • small anaphylatoxin C5a induces a variety of biological responses upon binding to the 7TM receptors C5aR and the C5L2, while the large C5b fragment nucleates formation of the membrane attack complex capable of killing susceptible pathogens by the formation of a pore structure
  • cell & other
    REGULATION
    inhibited by inactivated (cleaved) in the classical pathway by the complex C4b,C2a,C3b and in the alternative pathway by the complex C3b,BF
    ASSOCIATED DISORDERS
    corresponding disease(s) C5
    related resource C5base - Mutation registry for C5 deficiency
    Susceptibility
  • to hepatic fibrosis
  • to rheumatoid arthritis
  • to adverse cardiovascular outcome in male patients with carotid atherosclerosis
  • Variant & Polymorphism other
  • a common genetic variant at the TRAF1-C5 locus is associated with an increased risk of anti-CCP-positive rheumatoid arthritis (Plenge 2007)
  • C5 rs17611 GG genotype is associated with increased C5a plasma levels and represents a risk factor for adverse cardiovascular outcome in male patients with carotid atherosclerosis
  • Candidate gene
    Marker
  • C5 fragment levels in cerebrospinal fluid (CSF) of patients with bacterial meningitis correlated with several clinical indicators of poor prognosis
  • Therapy target
    SystemTypeDisorderPubmed
    immunologyinfectious 
    C5-specific monoclonal antibodies could be a promising new antiinflammatory adjuvant therapy for pneumococcal meningitis
    ANIMAL & CELL MODELS
  • C5a receptor-deficient mice with pneumococcal meningitis had lower CSF wbc counts and decreased brain damage compared with WT mice