complement component 5

Complement component 5 is the fifth component of complement, which plays an important role in inflammatory and cell killing processes. This protein is composed 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 C5-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.

The drug eculizumab (trade name Soliris) prevents cleavage of C5 into C5a and C5b.{{cite journal | title=Eculizumab| vauthors=Dubois E, Cohen A| journal=Br J Clin Pharmacol| year=2009| volume=68| pages=318–319| pmc=2766470 |doi=10.1111/j.1365-2125.2009.03491.x | issue=3 | pmid=19740388}}

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• {{cite journal | vauthors=Tack BF, Morris SC, Prahl JW |title=Fifth component of human complement: purification from plasma and polypeptide chain structure |journal=Biochemistry |volume=18 |issue= 8 |pages= 1490–7 |year= 1979 |pmid= 106884 |doi=10.1021/bi00575a016 }}
• {{cite journal | vauthors=Fernandez HN, Hugli TE |title=Primary structural analysis of the polypeptide portion of human C5a anaphylatoxin. Polypeptide sequence determination and assignment of the oligosaccharide attachment site in C5a |journal=J. Biol. Chem. |volume=253 |issue= 19 |pages= 6955–64 |year= 1978 |doi=10.1016/S0021-9258(17)38013-4 |pmid= 690134 |doi-access=free }}
• {{cite journal | author=DiScipio RG |title=Formation and structure of the C5b-7 complex of the lytic pathway of complement |journal=J. Biol. Chem. |volume=267 |issue= 24 |pages= 17087–94 |year= 1992 |doi=10.1016/S0021-9258(18)41897-2 |pmid= 1387399 |doi-access=free }}
• {{cite journal | vauthors=Haviland DL, Haviland JC, Fleischer DT |title=Complete cDNA sequence of human complement pro-C5. Evidence of truncated transcripts derived from a single copy gene |journal=J. Immunol. |volume=146 |issue= 1 |pages= 362–8 |year= 1991 |doi=10.4049/jimmunol.146.1.362 |pmid= 1984448 |display-authors=etal|doi-access=free }}
• {{cite journal | vauthors=Bohnsack JF, Mollison KW, Buko AM |title=Group B streptococci inactivate complement component C5a by enzymic cleavage at the C-terminus |journal=Biochem. J. |volume=273 |issue= Pt 3|pages= 635–40 |year= 1991 |pmid= 1996961 |doi= 10.1042/bj2730635| pmc=1149811 |display-authors=etal}}
• {{cite journal | vauthors=Lundwall AB, Wetsel RA, Kristensen T |title=Isolation and sequence analysis of a cDNA clone encoding the fifth complement component |journal=J. Biol. Chem. |volume=260 |issue= 4 |pages= 2108–12 |year= 1985 |doi=10.1016/S0021-9258(18)89523-0 |pmid= 2579066 |display-authors=etal|doi-access=free }}
• {{cite journal | vauthors=Zuiderweg ER, Fesik SW |title=Heteronuclear three-dimensional NMR spectroscopy of the inflammatory protein C5a |journal=Biochemistry |volume=28 |issue= 6 |pages= 2387–91 |year= 1989 |pmid= 2730871 |doi=10.1021/bi00432a008 }}
• {{cite journal | vauthors=Zuiderweg ER, Nettesheim DG, Mollison KW, Carter GW |title=Tertiary structure of human complement component C5a in solution from nuclear magnetic resonance data |journal=Biochemistry |volume=28 |issue= 1 |pages= 172–85 |year= 1989 |pmid= 2784981 |doi=10.1021/bi00427a025 }}
• {{cite journal | vauthors=Haefliger JA, Tschopp J, Vial N, Jenne DE |title=Complete primary structure and functional characterization of the sixth component of the human complement system. Identification of the C5b-binding domain in complement C6 |journal=J. Biol. Chem. |volume=264 |issue= 30 |pages= 18041–51 |year= 1989 |doi=10.1016/S0021-9258(19)84676-8 |pmid= 2808363 |doi-access=free }}
• {{cite journal | vauthors=Wetsel RA, Lemons RS, Le Beau MM |title=Molecular analysis of human complement component C5: localization of the structural gene to chromosome 9 |journal=Biochemistry |volume=27 |issue= 5 |pages= 1474–82 |year= 1988 |pmid= 3365401 |doi=10.1021/bi00405a012 |display-authors=etal}}
• {{cite journal | vauthors=Zuiderweg ER, Mollison KW, Henkin J, Carter GW |title=Sequence-specific assignments in the 1H NMR spectrum of the human inflammatory protein C5a |journal=Biochemistry |volume=27 |issue= 10 |pages= 3568–80 |year= 1988 |pmid= 3408713 |doi=10.1021/bi00410a007 }}
• {{cite journal | vauthors=Stewart JL, Kolb WP, Sodetz JM |title=Evidence that C5b recognizes and mediates C8 incorporation into the cytolytic complex of complement |journal=J. Immunol. |volume=139 |issue= 6 |pages= 1960–4 |year= 1987 |doi=10.4049/jimmunol.139.6.1960 |pmid= 3624872 |doi-access=free }}
• {{cite journal | vauthors=Buhl AM, Osawa S, Johnson GL |title=Mitogen-activated protein kinase activation requires two signal inputs from the human anaphylatoxin C5a receptor |journal=J. Biol. Chem. |volume=270 |issue= 34 |pages= 19828–32 |year= 1995 |pmid= 7649993 |doi=10.1074/jbc.270.34.19828 |doi-access=free }}
• {{cite journal | vauthors=Babkina IN, Seregin SV, Daniliuk NK |title=[Chemico-enzymatic synthesis, cloning and expression of a gene for an analog of human anaphylatoxin C5a] |journal=Bioorg. Khim. |volume=21 |issue= 5 |pages= 359–64 |year= 1995 |pmid= 7661861 |display-authors=etal}}
• {{cite journal | vauthors=Wang X, Fleischer DT, Whitehead WT |title=Inherited human complement C5 deficiency. Nonsense mutations in exons 1 (Gln1 to Stop) and 36 (Arg1458 to Stop) and compound heterozygosity in three African-American families |journal=J. Immunol. |volume=154 |issue= 10 |pages= 5464–71 |year= 1995 |doi=10.4049/jimmunol.154.10.5464 |pmid= 7730648 |display-authors=etal|doi-access=free }}
• {{cite journal | vauthors=Oppermann M, Götze O |title=Plasma clearance of the human C5a anaphylatoxin by binding to leucocyte C5a receptors |journal=Immunology |volume=82 |issue= 4 |pages= 516–21 |year= 1995 |pmid= 7835913 | pmc=1414921 }}
• {{cite journal | vauthors=Süsal C, Kirschfink M, Kröpelin M |title=Complement activation by recombinant HIV-1 glycoprotein gp120 |journal=J. Immunol. |volume=152 |issue= 12 |pages= 6028–34 |year= 1994 |doi=10.4049/jimmunol.152.12.6028 |pmid= 7911492 |display-authors=etal|doi-access=free }}
• {{cite journal | vauthors=Arribas J, Arizti P, Castaño JG |title=Antibodies against the C2 COOH-terminal region discriminate the active and latent forms of the multicatalytic proteinase complex |journal=J. Biol. Chem. |volume=269 |issue= 17 |pages= 12858–64 |year= 1994 |doi=10.1016/S0021-9258(18)99955-2 |pmid= 8175701 |doi-access=free }}
• {{cite journal | vauthors=Süsal C, Kirschfink M, Kröpelin M |title=Identification of complement activation sites in human immunodeficiency virus type-1 glycoprotein gp120 |journal=Blood |volume=87 |issue= 6 |pages= 2329–36 |year= 1996 |pmid= 8630395 |doi= 10.1182/blood.V87.6.2329.bloodjournal8762329|display-authors=etal|doi-access=free }}
• {{cite journal | vauthors=Ames RS, Li Y, Sarau HM |title=Molecular cloning and characterization of the human anaphylatoxin C3a receptor |journal=J. Biol. Chem. |volume=271 |issue= 34 |pages= 20231–4 |year= 1996 |pmid= 8702752 |doi=10.1074/jbc.271.34.20231 |display-authors=etal|doi-access=free }}
• {{cite journal | vauthors=Fredslund F, Laursen NS, Roversi P | title=Structure of and influence of a tick complement inhibitor on human complement component 5 | journal=Nat Immunol |volume= 9 |issue= 7 |pages= 753–60 |year= 2008 |pmid= 18536718 | doi=10.1038/ni.1625 | s2cid=205361576 |display-authors=etal}}

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• {{MeshName|Complement+5}}

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{{Complement system}}

Category:Complement system

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