The C1 complex (complement component 1, C1) is a protein complex involved in the complement system. It is the first component of the classical complement pathway and is composed of the subcomponents C1q, C1r and C1s.[1][2][3]
Structure
editThe C1 complex is ~790 kDa and is composed of 1 molecule of C1q, 2 molecules of C1r and 2 molecules of C1s, or C1qr2s2.[2][3][4]
Function
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Activation of the C1 complex initiates the classical complement pathway. This occurs when C1q binds to antigen-antibody complexes. The antibodies IgM or certain subclasses of IgG complexed with antigens are able to initiate the complement system: a single pentameric IgM can initiate the pathway, while several monomeric IgG molecules are needed.[3] C1q can also be activated in other ways, for example by binding to pentraxins such as C-reactive protein[2] or directly to the surface of pathogens.[1]
Such binding of C1q leads to conformational changes in the C1q molecule, which activates the associated C1r molecules. Active C1r cleaves the C1s molecules, activating them. Active C1s splits C4 and then C2, producing C4a, C4b, C2a and C2b. The classical pathway C3-convertase (C4bC2b complex) is created, which promotes cleavage of C3.[1]
References
edit- ^ a b c Janeway, CA Jr; Travers P; Walport M; et al. (2001). "The complement system and innate immunity". Immunobiology: The Immune System in Health and Disease. New York: Garland Science. Retrieved 29 December 2017.
- ^ a b c Abbas, Abul (2015). Cellular and molecular immunology. Philadelphia, PA: Elsevier Saunders. pp. 70–71. ISBN 978-0-323-28645-9.
- ^ a b c A., Owen, Judith (2013). Kuby immunology. Punt, Jenni., Stranford, Sharon A., Jones, Patricia P., Kuby, Janis. (7th ed.). New York: W.H. Freeman. pp. 190–194. ISBN 9781429219198. OCLC 820117219.
{{cite book}}: CS1 maint: multiple names: authors list (link) - ^ Venkatraman Girija, Umakhanth; Gingras, Alexandre R.; Marshall, Jamie E.; Panchal, Roshni; Sheikh, Md. Arif; Harper, James A. J.; Gál, Péter; Schwaeble, Wilhelm J.; Mitchell, Daniel A.; Moody, Peter C. E.; Wallis, Russell (20 August 2013). "Structural basis of the C1q/C1s interaction and its central role in assembly of the C1 complex of complement activation". Proceedings of the National Academy of Sciences. 110 (34): 13916–13920. Bibcode:2013PNAS..11013916V. doi:10.1073/pnas.1311113110. ISSN 0027-8424. PMC 3752233. PMID 23922389.
Further reading
editExternal links
edit- Complement C1 at the U.S. National Library of Medicine Medical Subject Headings (MeSH)