Complement component 5: Difference between revisions

Revision as of 07:24, 5 June 2011

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{{PBB|geneid=727}}

'''Complement component 5''' is a [[protein]] that in humans is encoded by the ''C5'' [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: complement component 5| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=727| accessdate = }}</ref>

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

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

* [[C5a]] plays an important role in [[chemotaxis]].<ref>{{GeorgiaImmunology|1/phagocyt}}</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 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 [[spasm|spasmogenic]] and [[chemotaxis|chemotactic]] activity, is derived from the alpha polypeptide via cleavage with a 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]] prevents cleavage of C5 into C5a and C5b.<ref>{{cite journal | title=Eculizumab| author=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}}</ref>

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

{{-}}

==References==

{{Reflist}}

<references/>

==Further reading==

{{refbegin | 2}}

{{PBB_Further_reading

| citations =

*{{cite journal | author=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 | author=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 |pmid= 690134 |doi= }}

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*{{cite journal | author=Süsal C, Kirschfink M, Kröpelin M, ''et al.'' |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= }}

*{{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}}

{{NLM content}}

{{gene-9-stub}}

{{PDB Gallery|geneid=727}}

{{Complement system}}

[[Category:Complement system]]

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[[de:Komplementkomponente C5]]