5hkj
From Proteopedia
(Difference between revisions)
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| - | '''Unreleased structure''' | ||
| - | + | ==Single Chain Recombinant Globular Head of the Complement System Protein C1q== | |
| - | + | <StructureSection load='5hkj' size='340' side='right' caption='[[5hkj]], [[Resolution|resolution]] 1.35Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[5hkj]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5HKJ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5HKJ FirstGlance]. <br> | |
| - | + | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr> | |
| - | [[ | + | <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[5hzf|5hzf]]</td></tr> |
| - | [[ | + | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5hkj FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5hkj OCA], [http://pdbe.org/5hkj PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5hkj RCSB], [http://www.ebi.ac.uk/pdbsum/5hkj PDBsum]</span></td></tr> |
| + | </table> | ||
| + | == Disease == | ||
| + | [[http://www.uniprot.org/uniprot/C1QA_HUMAN C1QA_HUMAN]] Defects in C1QA are a cause of complement component C1q deficiency (C1QD) [MIM:[http://omim.org/entry/613652 613652]]. A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis. | ||
| + | == Function == | ||
| + | [[http://www.uniprot.org/uniprot/C1QA_HUMAN C1QA_HUMAN]] C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes. | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
[[Category: Gaboriaud, C]] | [[Category: Gaboriaud, C]] | ||
| + | [[Category: Moreau, C P]] | ||
| + | [[Category: C1q]] | ||
| + | [[Category: Complement]] | ||
| + | [[Category: Gc1q domain]] | ||
| + | [[Category: Signaling protein]] | ||
Revision as of 15:29, 2 March 2016
Single Chain Recombinant Globular Head of the Complement System Protein C1q
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