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    C5 complement component 5 [ Homo sapiens (human) ]

    Gene ID: 727, updated on 19-May-2013
    Official Symbol
    C5provided by HGNC
    Official Full Name
    complement component 5provided by HGNC
    Primary source
    HGNC:1331
    See related
    Ensembl:ENSG00000106804; HPRD:00405; MIM:120900; Vega:OTTHUMG00000020579
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Homo sapiens
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
    Also known as
    C5a; C5b; CPAMD4
    Summary
    The protein encoded by this gene is the fifth component of complement, which plays an important role in inflammatory and cell killing processes. This protein is comprised of alpha and beta polypeptide chains that are linked by a disulfide bridge. An activation peptide, C5a, which is an anaphylatoxin that possesses potent spasmogenic and chemotactic activity, is derived from the alpha polypeptide via cleavage with a convertase. The C5b macromolecular cleavage product can form a complex with the C6 complement component, and this complex is the basis for formation of the membrane attack complex, which includes additional complement components. Mutations in this gene cause complement component 5 deficiency, a disease where patients show a propensity for severe recurrent infections. Defects in this gene have also been linked to a susceptibility to liver fibrosis and to rheumatoid arthritis. [provided by RefSeq, Jul 2008]
    Location :
    9q33-q34
    Sequence :
    Chromosome: 9; NC_000009.11 (123714613..123812554, complement)

    Chromosome 9 - NC_000009.11Genomic Context describing neighboring genes Neighboring gene PHD finger protein 19 Neighboring gene TNF receptor-associated factor 1 Neighboring gene centriolin Neighboring gene RAB14, member RAS oncogene family

    GeneRIFs: Gene References Into Functions What's a GeneRIF?

    Protein Gene Interaction Pubs
    Envelope surface glycoprotein gp120 env A synthetic peptide covering positions 233-251 of the HIV-1 gp120 protein binds to complement proteins C3, C4, C5, C9, and properdin PubMed
    env Complement proteins C4, C3d, C5b-9, and properdin bind to HIV-1 gp120-coated CD4+ T cells of healthy individuals when incubated in autologous serum PubMed

    Go to the HIV-1, Human Protein Interaction Database

    Products Interactant Other Gene Complex Source Pubs Description
    NP_001726.2 NP_001727.1 C5AR1    BIND  PubMed C5 (C5a) interacts with C5R1. 
    P01031 P06681 C2    HPRD  PubMed  
    P01031 P01024 C3    HPRD  PubMed  
    P01031 P01031 C5    HPRD  PubMed  
    P01031 P21730 C5AR1    HPRD  PubMed  
    P01031 P13671 C6    HPRD  PubMed  
    P01031 P10643 C7    HPRD  PubMed  
    P01031 P07358 C8B    HPRD  PubMed  
    P01031 P15169 CPN1    HPRD  PubMed  
    BioGRID:107188 BioGRID:107179 C3    BioGRID  PubMed Reconstituted Complex 
    BioGRID:107188 BioGRID:107189 C5AR1    BioGRID  PubMed Reconstituted Complex 
    BioGRID:107188 BioGRID:107190 C6    BioGRID  PubMed Co-fractionation; Reconstituted Complex 
    BioGRID:107188 BioGRID:107191 C7    BioGRID  PubMed Reconstituted Complex 
    BioGRID:107188 BioGRID:107193 C8B    BioGRID  PubMed Reconstituted Complex 
    BioGRID:107188 BioGRID:107753 CPB2    BioGRID  PubMed Biochemical Activity 
    BioGRID:107188 BioGRID:113164 UBC    BioGRID  PubMed Affinity Capture-MS 
    • Activation of C3 and C5, organism-specific biosystem (from REACTOME)
      Activation of C3 and C5, organism-specific biosystemThe 3 pathways of complement activation converge on the cleavage of C3 by C3 convertases. C3 convertase cleaves C3 into C3a and C3b - a central step of complement activation. C3a remains in the flu...
    • Class A/1 (Rhodopsin-like receptors), organism-specific biosystem (from REACTOME)
      Class A/1 (Rhodopsin-like receptors), organism-specific biosystemRhodopsin-like receptors (class A/1) are the largest group of GPCRs and are the best studied group from a functional and structural point of view. They show great diversity at the sequence level and ...
    • Complement Activation, Classical Pathway, organism-specific biosystem (from WikiPathways)
      Complement Activation, Classical Pathway, organism-specific biosystemThe complement system is a biochemical cascade that helps, or ???complements???, the ability of antibodies to clear pathogens from an organism. It is part of the immune system called the innate immun...
    • Complement and coagulation cascades, organism-specific biosystem (from KEGG)
      Complement and coagulation cascades, organism-specific biosystemThe complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation:...
    • Complement and coagulation cascades, conserved biosystem (from KEGG)
      Complement and coagulation cascades, conserved biosystemThe complement system is a proteolytic cascade in blood plasma and a mediator of innate immunity, a nonspecific defense mechanism against pathogens. There are three pathways of complement activation:...
    • Complement cascade, organism-specific biosystem (from REACTOME)
      Complement cascade, organism-specific biosystemThe complement system is a biochemical cascade, so named because it 'complements' the ability of antibodies to clear pathogens. It is part of the innate immune system. Complement system proteins circ...
    • G alpha (i) signalling events, organism-specific biosystem (from REACTOME)
      G alpha (i) signalling events, organism-specific biosystemThe classical signalling mechanism for G alpha (i) is inhibition of the cAMP dependent pathway through inhibition of adenylate cyclase. Decreased production of cAMP from ATP results in decreased act...
    • GPCR downstream signaling, organism-specific biosystem (from REACTOME)
      GPCR downstream signaling, organism-specific biosystemG protein-coupled receptors (GPCRs) are classically defined as the receptor, G-protein and downstream effectors, the alpha subunit of the G-protein being the primary signaling molecule. However, it h...
    • GPCR ligand binding, organism-specific biosystem (from REACTOME)
      GPCR ligand binding, organism-specific biosystemThere are more than 800 G-protein coupled receptor (GPCRs) in the human genome, making it the largest receptor superfamily. GPCRs are also the largest class of drug targets, involved in virtually all...
    • Herpes simplex infection, organism-specific biosystem (from KEGG)
      Herpes simplex infection, organism-specific biosystemHerpes simplex virus (HSV) infections are very common worldwide, with the prevalence of HSV-1 reaching up to 80%-90%. Primary infection with HSV takes place in the mucosa, followed by the establishme...
    • Herpes simplex infection, conserved biosystem (from KEGG)
      Herpes simplex infection, conserved biosystemHerpes simplex virus (HSV) infections are very common worldwide, with the prevalence of HSV-1 reaching up to 80%-90%. Primary infection with HSV takes place in the mucosa, followed by the establishme...
    • Immune System, organism-specific biosystem (from REACTOME)
      Immune System, organism-specific biosystemHumans are exposed to millions of potential pathogens daily, through contact, ingestion, and inhalation. Our ability to avoid infection depends on the adaptive immune system and during the first crit...
    • Innate Immune System, organism-specific biosystem (from REACTOME)
      Innate Immune System, organism-specific biosystemInnate immunity encompases the nonspecific part of immunity tha are part of an individual's natural biologic makeup
    • Peptide ligand-binding receptors, organism-specific biosystem (from REACTOME)
      Peptide ligand-binding receptors, organism-specific biosystemThese receptors, a subset of the Class A/1 (Rhodopsin-like) family, all bind peptide ligands which include the chemokines, opioids and somatostatins.
    • Pertussis, organism-specific biosystem (from KEGG)
      Pertussis, organism-specific biosystemPertussis, also known as whooping cough, is an acute respiratory infectious disease caused by a bacteria called Bordetella Pertussis. The characteristic symptoms are paroxysmal cough, inspiratory whe...
    • Pertussis, conserved biosystem (from KEGG)
      Pertussis, conserved biosystemPertussis, also known as whooping cough, is an acute respiratory infectious disease caused by a bacteria called Bordetella Pertussis. The characteristic symptoms are paroxysmal cough, inspiratory whe...
    • Prion diseases, organism-specific biosystem (from KEGG)
      Prion diseases, organism-specific biosystemPrion diseases, also termed transmissible spongiform encephalopathies (TSEs), are a group of fatal neurodegenerative diseases that affect humans and a number of other animal species. The etiology of ...
    • Prion diseases, conserved biosystem (from KEGG)
      Prion diseases, conserved biosystemPrion diseases, also termed transmissible spongiform encephalopathies (TSEs), are a group of fatal neurodegenerative diseases that affect humans and a number of other animal species. The etiology of ...
    • Signal Transduction, organism-specific biosystem (from REACTOME)
      Signal Transduction, organism-specific biosystemSignal transduction is a process in which extracellular signals elicit changes in cell state and activity. Transmembrane receptors sense changes in the cellular environment by binding ligands, such a...
    • Signaling by GPCR, organism-specific biosystem (from REACTOME)
      Signaling by GPCR, organism-specific biosystemG protein-coupled receptors (GPCRs; 7TM receptors; seven transmembrane domain receptors; heptahelical receptors; G protein-linked receptors [GPLR]) are the largest family of transmembrane receptors i...
    • Staphylococcus aureus infection, organism-specific biosystem (from KEGG)
      Staphylococcus aureus infection, organism-specific biosystemStaphylococcus aureus can cause multiple forms of infections ranging from superficial skin infections to food poisoning and life-threatening infections. The organism has several ways to divert the ef...
    • Staphylococcus aureus infection, conserved biosystem (from KEGG)
      Staphylococcus aureus infection, conserved biosystemStaphylococcus aureus can cause multiple forms of infections ranging from superficial skin infections to food poisoning and life-threatening infections. The organism has several ways to divert the ef...
    • Systemic lupus erythematosus, organism-specific biosystem (from KEGG)
      Systemic lupus erythematosus, organism-specific biosystemSystemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterised by the production of IgG autoantibodies that are specific for self-antigens, such as DNA, nuclear proteins and cert...
    • Systemic lupus erythematosus, conserved biosystem (from KEGG)
      Systemic lupus erythematosus, conserved biosystemSystemic lupus erythematosus (SLE) is a prototypic autoimmune disease characterised by the production of IgG autoantibodies that are specific for self-antigens, such as DNA, nuclear proteins and cert...
    • Terminal pathway of complement, organism-specific biosystem (from REACTOME)
      Terminal pathway of complement, organism-specific biosystemAfter cleavage of C5, C5b undergoes conformational changes and exposes a binding site for C6. C5b6 binds C7 resulting in the exposure of membrane binding sites and incorporation into target membranes...

    Markers

    Homology

    Clone Names

    • FLJ17816, FLJ17822, MGC142298

    Gene Ontology Provided by GOA

    Function Evidence Code Pubs
    C5a anaphylatoxin chemotactic receptor binding IEA
    Inferred from Electronic Annotation
    more info
     
    chemokine activity TAS
    Traceable Author Statement
    more info
    PubMed 
    endopeptidase inhibitor activity IEA
    Inferred from Electronic Annotation
    more info
     
    receptor binding TAS
    Traceable Author Statement
    more info
    PubMed 
    Process Evidence Code Pubs
    G-protein coupled receptor signaling pathway TAS
    Traceable Author Statement
    more info
    PubMed 
    activation of MAPK activity TAS
    Traceable Author Statement
    more info
    PubMed 
    cell surface receptor signaling pathway TAS
    Traceable Author Statement
    more info
    PubMed 
    cellular calcium ion homeostasis IEA
    Inferred from Electronic Annotation
    more info
     
    chemotaxis IEA
    Inferred from Electronic Annotation
    more info
     
    complement activation TAS
    Traceable Author Statement
    more info
     
    complement activation, alternative pathway IEA
    Inferred from Electronic Annotation
    more info
     
    complement activation, classical pathway IEA
    Inferred from Electronic Annotation
    more info
     
    cytolysis IEA
    Inferred from Electronic Annotation
    more info
     
    glucose homeostasis IEA
    Inferred from Electronic Annotation
    more info
     
    in utero embryonic development IEA
    Inferred from Electronic Annotation
    more info
     
    induction of apoptosis IEA
    Inferred from Electronic Annotation
    more info
     
    inflammatory response TAS
    Traceable Author Statement
    more info
    PubMed 
    innate immune response TAS
    Traceable Author Statement
    more info
     
    leukocyte migration involved in inflammatory response IEA
    Inferred from Electronic Annotation
    more info
     
    negative regulation of dopamine secretion IEA
    Inferred from Electronic Annotation
    more info
     
    negative regulation of macrophage chemotaxis IDA
    Inferred from Direct Assay
    more info
    PubMed 
    negative regulation of norepinephrine secretion IEA
    Inferred from Electronic Annotation
    more info
     
    positive regulation of angiogenesis IEA
    Inferred from Electronic Annotation
    more info
     
    positive regulation of chemokine secretion IDA
    Inferred from Direct Assay
    more info
    PubMed 
    positive regulation of chemotaxis IEA
    Inferred from Electronic Annotation
    more info
     
    positive regulation vascular endothelial growth factor production IDA
    Inferred from Direct Assay
    more info
    PubMed 
    response to stress TAS
    Traceable Author Statement
    more info
    PubMed 
    Component Evidence Code Pubs
    extracellular region TAS
    Traceable Author Statement
    more info
     
    extracellular space IEA
    Inferred from Electronic Annotation
    more info
     
    membrane attack complex IEA
    Inferred from Electronic Annotation
    more info
     
    Preferred Names
    complement C5
    Names
    complement C5
    prepro-C5
    C5a anaphylatoxin
    anaphylatoxin C5a analog
    C3 and PZP-like alpha-2-macroglobulin domain-containing protein 4

    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    Genomic

    1. NG_007364.1 RefSeqGene

      Range
      5001..102942
      Download
      GenBank, FASTA, Sequence Viewer (Graphics), LRG_28

    mRNA and Protein(s)

    1. NM_001735.2NP_001726.2  complement C5 preproprotein

      Status: REVIEWED

      Source sequence(s)
      AV682721, BC022299, BG533927, CB250401, M57729, M65134, T82068
      Consensus CDS
      CCDS6826.1
      UniProtKB/Swiss-Prot
      P01031
      Related
      ENSP00000223642, OTTHUMP00000022001, ENST00000223642, OTTHUMT00000053844
      Conserved Domains (9) summary
      cd00017
      Location:682750
      Blast Score: 229
      ANATO; Anaphylatoxin homologous domain; C3a, C4a and C5a anaphylatoxins are protein fragments generated enzymatically in serum during activation of complement molecules C3, C4, and C5. They induce smooth muscle contraction. These fragments are homologous to ...
      cd02896
      Location:9931300
      Blast Score: 807
      complement_C3_C4_C5; Proteins similar to C3, C4 and C5 of vertebrate complement. The vertebrate complement system, comprised of a large number of distinct plasma proteins, is an effector of both the acquired and innate immune systems. The point of convergence of the ...
      cd03582
      Location:15271676
      Blast Score: 650
      NTR_complement_C5; NTR/C345C domain, complement C5 subfamily; The NTR domain found in complement C5 is also known as C345C because it occurs at the C-terminus of complement C3, C4 and C5. Complement C5 is activated by C5 convertase, which itself is a complex between C3b ...
      pfam00207
      Location:772859
      Blast Score: 302
      A2M; Alpha-2-macroglobulin family
      pfam01835
      Location:126219
      Blast Score: 211
      A2M_N; MG2 domain
      pfam07677
      Location:14221509
      Blast Score: 284
      A2M_recep; A-macroglobulin receptor
      pfam07678
      Location:10511302
      Blast Score: 659
      A2M_comp; A-macroglobulin complement component
      pfam07703
      Location:472610
      Blast Score: 281
      A2M_N_2; Alpha-2-macroglobulin family N-terminal region
      pfam11974
      Location:22111
      Blast Score: 127
      MG1; Alpha-2-macroglobulin MG1 domain

    RefSeqs of Annotated Genomes: Homo sapiens Annotation Release 104

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCh37.p10 Primary Assembly

    Genomic

    1. NC_000009.11 Reference GRCh37.p10 Primary Assembly

      Range
      123714613..123812554, complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Alternate HuRef

    Genomic

    1. AC_000141.1 Alternate HuRef

      Range
      93332095..93430452, complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    Alternate CHM1_1.0

    Genomic

    1. NC_018920.1 Alternate CHM1_1.0

      Range
      123726947..123824901, complement
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

      Supplemental Content

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