Complement component 2: Difference between revisions

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{{Infobox_gene}}
{{Infobox_gene}}
'''Complement C2''' is a [[protein]] that in humans is encoded by the ''C2'' [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: C2 complement component 2| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=717| accessdate = }}</ref> The protein encoded by this gene is part of the classical pathway of [[complement system]], acting as a multi-domain serine protease. Deficiency of C2 has been associated with certain autoimmune diseases.<ref name="entrez"/>
'''Complement C2''' is a [[protein]] that in humans is encoded by the ''C2'' [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: C2 complement component 2| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=717| accessdate = }}</ref> The protein encoded by this gene is part of the [[classical complement pathway|classical pathway]] of the [[complement system]], acting as a multi-domain serine protease. Deficiency of C2 has been associated with certain autoimmune diseases.<ref name="entrez"/>


== Function ==
== Function ==
In the [[classical complement pathway|classical]] and [[lectin pathway|lectin]] pathways of [[complement activation]], formation of the [[C3-convertase]] and [[C5-convertase]]s requires binding of C2 to an activated surface-bound [[C4b]] in the presence of [[magnesium|Mg<sup>2+</sup>]]; the resultant C4bC2 complex is then cleaved by [[C1s]] or [[MASP2 (protein)|MASP2]] into C2a and C2b. It is thought that cleavage of C2 by C1s, while bound to C4b, results in a conformational rotation of C2b whereas the released C2a fragment may retain most of its original structure.


In the classical and [[lectin]] pathways of [[complement activation]], formation of the [[C3-convertase]] and [[C5-convertase]]s requires binding of C2 to an activated surface-bound [[C4b]] in the presence of [[magnesium|Mg<sup>2+</sup>]]; the resultant C4bC2 complex is then cleaved by [[C1s]] or [[MASP2 (protein)|MASP2]] into C2a and C2b. It is thought that cleavage of C2 by C1s, while bound to C4b. results into a conformational rotation of C2a whereas the released C2b fragment may retain most of its original structure. C2a is the larger, enzymatically active fragment which is incorporated into the C3 convertase in this pathway, C4b2a. C2b is released into the fluid phase.<ref name="c2bstructure">{{cite journal | vauthors = Takeuchi E, Doi T, Shimada T, Muso E, Maruyama N, Yoshida H | title = Retroviral gp70 antigen in spontaneous mesangial glomerulonephritis of ddY mice | journal = Kidney International | volume = 35 | issue = 2 | pages = 638–46 | date = Feb 1989 | pmid = 2651757 | doi=10.1038/ki.1989.33 }}</ref>
C2b is the larger, enzymatically active fragment which is incorporated into the C3 convertase in this pathway, C4b2b (NB: [[Complement system#Complement protein fragment nomenclature|some sources now refer]] to the larger fragment of C2 as C2b, making the C3 convertase C4b2b, whereas older sources refer to the larger fragment of C2 as C2a, making the C3 convertase C4b2a). The smaller fragment, C2a (or C2b, depending on the source) is released into the fluid phase.<ref name="c2 fragment structure">{{cite journal | vauthors = Krishnan V, Xu Y, Macon K, Volanakis JE, Narayana SV | title = The structure of C2b, a fragment of complement component C2 produced during C3 convertase formation | journal = Acta Crystallographica D | volume = 65 | issue = Pt 3 | pages = 266–74 | year = 2009 | pmid = 19237749 | pmc = 2651757 | doi = 10.1107/S0907444909000389 }}</ref>
==Deficiency==
The deficiency of C2 and C4 together lead to severe cases of gonorrhea and meningococcal diseases leading to stupor and death


== References ==
== References ==

Latest revision as of 17:03, 27 December 2018

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Identifiers
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External IDsGeneCards: [1]
Orthologs
SpeciesHumanMouse
Entrez

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Ensembl

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UniProt

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Wikidata
View/Edit Human

Complement C2 is a protein that in humans is encoded by the C2 gene.[1] The protein encoded by this gene is part of the classical pathway of the complement system, acting as a multi-domain serine protease. Deficiency of C2 has been associated with certain autoimmune diseases.[1]

Function

In the classical and lectin pathways of complement activation, formation of the C3-convertase and C5-convertases requires binding of C2 to an activated surface-bound C4b in the presence of Mg2+; the resultant C4bC2 complex is then cleaved by C1s or MASP2 into C2a and C2b. It is thought that cleavage of C2 by C1s, while bound to C4b, results in a conformational rotation of C2b whereas the released C2a fragment may retain most of its original structure.

C2b is the larger, enzymatically active fragment which is incorporated into the C3 convertase in this pathway, C4b2b (NB: some sources now refer to the larger fragment of C2 as C2b, making the C3 convertase C4b2b, whereas older sources refer to the larger fragment of C2 as C2a, making the C3 convertase C4b2a). The smaller fragment, C2a (or C2b, depending on the source) is released into the fluid phase.[2]

References

  1. ↑ 1.0 1.1 "Entrez Gene: C2 complement component 2".
  2. ↑ Krishnan V, Xu Y, Macon K, Volanakis JE, Narayana SV (2009). "The structure of C2b, a fragment of complement component C2 produced during C3 convertase formation". Acta Crystallographica D. 65 (Pt 3): 266–74. doi:10.1107/S0907444909000389. PMC 2651757. PMID 19237749.

Further reading

External links


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