Complement C5
From Proteopedia
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<StructureSection load='3pvm' size='340' side='right' caption='Human complement C5 complex (grey and pink) with cobra venom factor (green and yellow) (PDB code [[3pvm]])' scene=''> | <StructureSection load='3pvm' size='340' side='right' caption='Human complement C5 complex (grey and pink) with cobra venom factor (green and yellow) (PDB code [[3pvm]])' scene=''> | ||
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== Function == | == Function == | ||
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+ | '''Complement C5''' (CC5) is the fifth component of the complement system. The complement system is part of the innate immune system. CC5 is composed of α chain (C5a) and β chain (C5b). Proteoplytic degradation of CC5 produces '''anaphylatoxin''' which is a mediator of local inflammatory process. The C terminal domain of CC5 (residues 1530-1676) is homologous to a domain in netrins and is named '''C345C'''. Activation of CC5 by C5 convertase initiates the assembly of the C5 to C9 components into the membrane attack complex. <ref>PMID:18536718</ref> | ||
== Disease == | == Disease == | ||
- | Mutations in CC5 cause a deficiency leading to severe recurrent infections and are linked to liver fibrosis and rheumatoid arthritis. | + | Mutations in CC5 cause a deficiency leading to severe recurrent infections and are linked to liver fibrosis, Leiner's disease and rheumatoid arthritis. |
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</StructureSection> | </StructureSection> | ||
Revision as of 07:50, 14 December 2015
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3D Structures of complement C5
Updated on 14-December-2015
References
- ↑ Fredslund F, Laursen NS, Roversi P, Jenner L, Oliveira CL, Pedersen JS, Nunn MA, Lea SM, Discipio R, Sottrup-Jensen L, Andersen GR. Structure of and influence of a tick complement inhibitor on human complement component 5. Nat Immunol. 2008 Jul;9(7):753-60. Epub 2008 Jun 8. PMID:18536718 doi:10.1038/ni.1625