Complement component 5: Difference between revisions

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{{Short description|Protein found in humans}}

'''Complement component 5''' is a protein involved in the [[complement system]].

{{Infobox_gene}}

'''Complement component 5''' is a [[protein]] that in humans is encoded by the ''C5'' [[gene]].<ref name="entrez">{{cite web | title = C5 complement C5 (Homo sapiens (human)) Gene ID: 727 |date= 29 November 2020| url = https://www.ncbi.nlm.nih.gov/gene?Db=gene&Cmd=ShowDetailView&TermToSearch=727 |website=www.ncbi.nlm.nih.gov |access-date=9 December 2020}}</ref>

Complement component 5 is involved in the [[complement system]]. It is cleaved into [[Complement component 5a|C5a]] and C5b:

* [[Complement component 5a|C5a]] plays an important role in [[chemotaxis]].<ref>{{GeorgiaImmunology|1/phagocyt}}{{Deadlink|date=December 2020}}</ref>

* C5b forms the first part of the [[complement membrane attack complex]].

Deficiency is thought to cause [[Leiner's disease]].

== Function ==

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 [[spasm]]ogenic and [[chemotaxis|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 [[complement component 6|C6 complement component]], and this complex is the basis for formation of the membrane attack complex, which includes additional complement components.<ref name="entrez"/>

== Clinical significance ==

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]].<ref name="entrez"/>

== Therapeutic applications ==

The drug [[eculizumab]] (trade name '''Soliris''') prevents cleavage of C5 into C5a and C5b.<ref>{{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}}</ref>

== Complement system pathway ==

[[Image:Formowanie MACFormowanie_MAC-en.svg|thumb|left|400px|left|Membrane attack complex. Some labels are in Polish.]]

{{-}}

==References==

{{Reflist}}

<references/>

==Further reading==

{{refbegin | 2}}

*{{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 }}

{{PBB_Further_reading

*{{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 }}

| citations =

*{{cite journal | author=TackDiScipio BF, Morris SC, Prahl JWRG |title=FifthFormation componentand structure of humanthe complement:C5b-7 purificationcomplex fromof plasmathe andlytic polypeptidepathway chainof structure.complement |journal=BiochemistryJ. Biol. Chem. |volume=18267 |issue= 824 |pages= 1490–717087–94 |year= 19791992 |doi=10.1016/S0021-9258(18)41897-2 |pmid= 1068841387399 |doi-access=10.1021/bi00575a016 free }}

*{{cite journal | authorvauthors=FernandezHaviland HNDL, HugliHaviland TEJC, Fleischer DT |title=PrimaryComplete structuralcDNA analysis of the polypeptide portionsequence of human C5acomplement anaphylatoxinpro-C5. PolypeptideEvidence sequenceof determinationtruncated andtranscripts assignmentderived offrom thea oligosaccharidesingle attachmentcopy site in C5a.gene |journal=J. Biol. ChemImmunol. |volume=253146 |issue= 191 |pages= 6955–64362–8 |year= 19781991 |doi=10.4049/jimmunol.146.1.362 |pmid= 6901341984448 |display-authors=etal|doi-access= free }}

*{{cite journal | authorvauthors=DiScipioBohnsack RGJF, Mollison KW, Buko AM |title=FormationGroup andB structurestreptococci ofinactivate thecomplement C5b-7component complexC5a ofby theenzymic lyticcleavage pathwayat ofthe complement.C-terminus |journal=JBiochem. Biol. ChemJ. |volume=267273 |issue= 24 Pt 3|pages= 17087–94635–40 |year= 19921991 |pmid= 13873991996961 |doi= 10.1042/bj2730635| pmc=1149811 |display-authors=etal}}

*{{cite journal | authorvauthors=HavilandLundwall DLAB, HavilandWetsel JCRA, FleischerKristensen DT, ''et al.''T |title=CompleteIsolation cDNAand sequence of human complement pro-C5. Evidenceanalysis of truncateda transcriptscDNA derivedclone fromencoding athe singlefifth copycomplement gene.component |journal=J. ImmunolBiol. Chem. |volume=146260 |issue= 14 |pages= 362–82108–12 |year= 19911985 |doi=10.1016/S0021-9258(18)89523-0 |pmid= 19844482579066 |display-authors=etal|doi-access= free }}

*{{cite journal | authorvauthors=BohnsackZuiderweg JFER, MollisonFesik KW, Buko AM, ''et al.''SW |title=GroupHeteronuclear Bthree-dimensional streptococciNMR inactivatespectroscopy complementof componentthe C5ainflammatory byprotein enzymic cleavage at the C-terminus.C5a |journal=Biochem. J.Biochemistry |volume=273 ( Pt 3)28 |issue= 6 |pages= 635–402387–91 |year= 19911989 |pmid= 19969612730871 |doi=10.1021/bi00432a008 }}

*{{cite journal | authorvauthors=LundwallZuiderweg ABER, WetselNettesheim RADG, KristensenMollison TKW, ''etCarter al.''GW |title=IsolationTertiary andstructure sequenceof analysishuman ofcomplement acomponent cDNAC5a clonein encodingsolution thefrom fifthnuclear complementmagnetic component.resonance data |journal=J. Biol. Chem.Biochemistry |volume=26028 |issue= 41 |pages= 2108–12172–85 |year= 19851989 |pmid= 25790662784981 |doi=10.1021/bi00427a025 }}

*{{cite journal | authorvauthors=ZuiderwegHaefliger ERJA, FesikTschopp SWJ, Vial N, Jenne DE |title=HeteronuclearComplete three-dimensionalprimary NMRstructure spectroscopyand functional characterization of the inflammatorysixth proteincomponent C5aof the human complement system. Identification of the C5b-binding ___domain in complement C6 |journal=BiochemistryJ. Biol. Chem. |volume=28264 |issue= 630 |pages= 2387–9118041–51 |year= 1989 |doi=10.1016/S0021-9258(19)84676-8 |pmid= 27308712808363 |doi-access=10.1021/bi00432a008 free }}

*{{cite journal | authorvauthors=ZuiderwegWetsel ERRA, NettesheimLemons DGRS, MollisonLe KW, CarterBeau GWMM |title=TertiaryMolecular structureanalysis of human complement component C5aC5: inlocalization solutionof fromthe nuclearstructural magneticgene resonanceto data.chromosome 9 |journal=Biochemistry |volume=2827 |issue= 15 |pages= 172–851474–82 |year= 19891988 |pmid= 27849813365401 |doi=10.1021/bi00427a025bi00405a012 |display-authors=etal}}

*{{cite journal | authorvauthors=HaefligerZuiderweg JAER, TschoppMollison JKW, VialHenkin NJ, JenneCarter DEGW |title=CompleteSequence-specific primaryassignments structurein andthe functional characterization of the1H sixthNMR componentspectrum of the human complementinflammatory system.protein Identification of the C5b-binding ___domain in complement C6.C5a |journal=J. Biol. Chem.Biochemistry |volume=26427 |issue= 3010 |pages= 18041–513568–80 |year= 19891988 |pmid= 28083633408713 |doi=10.1021/bi00410a007 }}

*{{cite journal | authorvauthors=WetselStewart RAJL, LemonsKolb RSWP, LeSodetz Beau MM, ''et al.''JM |title=MolecularEvidence analysisthat ofC5b humanrecognizes complementand componentmediates C5:C8 localizationincorporation ofinto the structuralcytolytic genecomplex toof chromosome 9.complement |journal=BiochemistryJ. Immunol. |volume=27139 |issue= 56 |pages= 1474–821960–4 |year= 19881987 |doi=10.4049/jimmunol.139.6.1960 |pmid= 33654013624872 |doi-access=10.1021/bi00405a012 free }}

*{{cite journal | authorvauthors=ZuiderwegBuhl ERAM, MollisonOsawa KWS, HenkinJohnson J, Carter GWGL |title=SequenceMitogen-specificactivated assignmentsprotein inkinase theactivation 1Hrequires NMRtwo spectrumsignal ofinputs from the human inflammatory proteinanaphylatoxin C5a. receptor |journal=BiochemistryJ. Biol. Chem. |volume=27270 |issue= 1034 |pages= 3568–8019828–32 |year= 19881995 |pmid= 34087137649993 |doi=10.10211074/bi00410a007jbc.270.34.19828 |doi-access=free }}

*{{cite journal | authorvauthors=StewartBabkina JLIN, KolbSeregin WPSV, SodetzDaniliuk JMNK |title=Evidence[Chemico-enzymatic thatsynthesis, C5b recognizescloning and mediatesexpression C8of incorporationa intogene thefor cytolytican complexanalog of complement.human anaphylatoxin C5a] |journal=JBioorg. ImmunolKhim. |volume=13921 |issue= 65 |pages= 1960–4359–64 |year= 19871995 |pmid= 36248727661861 |doidisplay-authors= etal}}

*{{cite journal | authorvauthors=BuhlWang AMX, OsawaFleischer SDT, JohnsonWhitehead GLWT |title=Mitogen-activatedInherited proteinhuman kinasecomplement activationC5 requiresdeficiency. twoNonsense signalmutations inputsin fromexons the1 human(Gln1 anaphylatoxinto C5aStop) receptor.and 36 (Arg1458 to Stop) and compound heterozygosity in three African-American families |journal=J. Biol. ChemImmunol. |volume=270154 |issue= 3410 |pages= 19828–325464–71 |year= 1995 |pmid= 7649993 |doi=10.10744049/jbcjimmunol.270154.3410.198285464 |pmid= 7730648 |display-authors=etal|doi-access=free }}

*{{cite journal | authorvauthors=BabkinaOppermann INM, SereginGötze SV, Daniliuk NK, ''et al.''O |title=[Chemico-enzymatic synthesis, cloning andPlasma expressionclearance of athe genehuman forC5a ananaphylatoxin analogby ofbinding humanto anaphylatoxinleucocyte C5a] receptors |journal=Bioorg. Khim.Immunology |volume=2182 |issue= 54 |pages= 359–64516–21 |year= 1995 |pmid= 76618617835913 |doi pmc=1414921 }}

*{{cite journal | authorvauthors=WangSüsal XC, FleischerKirschfink DTM, WhiteheadKröpelin WT, ''et al.''M |title=InheritedComplement humanactivation complementby C5 deficiency. Nonsense mutations in exonsrecombinant HIV-1 (Gln1glycoprotein to Stop) and 36 (Arg1458 to Stop) and compound heterozygosity in three African-American families.gp120 |journal=J. Immunol. |volume=154152 |issue= 1012 |pages= 5464–716028–34 |year= 19951994 |doi=10.4049/jimmunol.152.12.6028 |pmid= 77306487911492 |display-authors=etal|doi-access= free }}

*{{cite journal | authorvauthors=OppermannArribas MJ, GötzeArizti OP, Castaño JG |title=PlasmaAntibodies clearanceagainst ofthe C2 COOH-terminal region discriminate the humanactive C5aand anaphylatoxinlatent byforms bindingof tothe leucocytemulticatalytic C5aproteinase receptors.complex |journal=ImmunologyJ. Biol. Chem. |volume=82269 |issue= 417 |pages= 516–2112858–64 |year= 19951994 |doi=10.1016/S0021-9258(18)99955-2 |pmid= 78359138175701 |doi-access= free }}

*{{cite journal | authorvauthors=Süsal C, Kirschfink M, Kröpelin M, ''et al.'' |title=ComplementIdentification of complement activation bysites in human immunodeficiency recombinantvirus HIVtype-1 glycoprotein gp120. |journal=J. Immunol.Blood |volume=15287 |issue= 126 |pages= 6028–342329–36 |year= 19941996 |pmid= 79114928630395 |doi= 10.1182/blood.V87.6.2329.bloodjournal8762329|display-authors=etal|doi-access=free }}

*{{cite journal | authorvauthors=ArribasAmes JRS, AriztiLi PY, CastañoSarau JGHM |title=AntibodiesMolecular against the C2 COOH-terminal region discriminate the activecloning and latent formscharacterization of the multicatalytichuman proteinaseanaphylatoxin complex.C3a receptor |journal=J. Biol. Chem. |volume=269271 |issue= 1734 |pages= 12858–6420231–4 |year= 19941996 |pmid= 81757018702752 |doi=10.1074/jbc.271.34.20231 |display-authors=etal|doi-access=free }}

*{{cite journal | authorvauthors=SüsalFredslund CF, KirschfinkLaursen MNS, KröpelinRoversi M,P ''et| al.'' |title=IdentificationStructure of complementand activationinfluence sitesof ina humantick immunodeficiencycomplement virusinhibitor type-1on glycoproteinhuman gp120.complement component 5 | journal=BloodNat Immunol |volume=87 9 |issue= 67 |pages= 2329–36753–60 |year= 19962008 |pmid= 863039518536718 | doi=10.1038/ni.1625 | s2cid=205361576 |display-authors=etal}}

*{{cite journal | author=Ames RS, Li Y, Sarau HM, ''et al.'' |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 }}

}}

*{{cite journal | author=Fredslund F, Laursen NS, Roversi P ''et al.'' | 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 }}

{{refend}}

{{PDB Gallery|geneid=727}}

[[Image:Formowanie MAC.svg|thumb|400px|left|Membrane attack complex. Some labels are in Polish.]]

==External links==

* {{MeshName|Complement+5}}

{{gene-9-stubNLM content}}

{{PBBPDB Gallery|geneid=727}}

{{Complement system}}

[[Category:Complement system]]

{{Gene-9-stub}}

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