1pk6
From Proteopedia
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==Globular Head of the Complement System Protein C1q== | ==Globular Head of the Complement System Protein C1q== | ||
| - | <StructureSection load='1pk6' size='340' side='right' caption='[[1pk6]], [[Resolution|resolution]] 1.85Å' scene=''> | + | <StructureSection load='1pk6' size='340' side='right'caption='[[1pk6]], [[Resolution|resolution]] 1.85Å' scene=''> |
== Structural highlights == | == Structural highlights == | ||
| - | <table><tr><td colspan='2'>[[1pk6]] is a 3 chain structure with sequence from [ | + | <table><tr><td colspan='2'>[[1pk6]] is a 3 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1PK6 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1PK6 FirstGlance]. <br> |
| - | </td></tr><tr id=' | + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.85Å</td></tr> |
| - | <tr id=' | + | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene></td></tr> |
| - | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[ | + | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1pk6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1pk6 OCA], [https://pdbe.org/1pk6 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1pk6 RCSB], [https://www.ebi.ac.uk/pdbsum/1pk6 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1pk6 ProSAT]</span></td></tr> |
</table> | </table> | ||
== Disease == | == Disease == | ||
| - | [ | + | [https://www.uniprot.org/uniprot/C1QA_HUMAN C1QA_HUMAN] Defects in C1QA are a cause of complement component C1q deficiency (C1QD) [MIM:[https://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 == | == Function == | ||
| - | [ | + | [https://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. |
== Evolutionary Conservation == | == Evolutionary Conservation == | ||
[[Image:Consurf_key_small.gif|200px|right]] | [[Image:Consurf_key_small.gif|200px|right]] | ||
Check<jmol> | Check<jmol> | ||
<jmolCheckbox> | <jmolCheckbox> | ||
| - | <scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/pk/1pk6_consurf.spt"</scriptWhenChecked> | + | <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/pk/1pk6_consurf.spt"</scriptWhenChecked> |
| - | <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/ | + | <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview03.spt</scriptWhenUnchecked> |
<text>to colour the structure by Evolutionary Conservation</text> | <text>to colour the structure by Evolutionary Conservation</text> | ||
</jmolCheckbox> | </jmolCheckbox> | ||
| - | </jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/ | + | </jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1pk6 ConSurf]. |
<div style="clear:both"></div> | <div style="clear:both"></div> | ||
<div style="background-color:#fffaf0;"> | <div style="background-color:#fffaf0;"> | ||
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | ||
</div> | </div> | ||
| + | <div class="pdbe-citations 1pk6" style="background-color:#fffaf0;"></div> | ||
== References == | == References == | ||
<references/> | <references/> | ||
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</StructureSection> | </StructureSection> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
| - | [[Category: Arlaud | + | [[Category: Large Structures]] |
| - | [[Category: Darnault | + | [[Category: Arlaud GJ]] |
| - | [[Category: Fontecilla-Camps | + | [[Category: Darnault C]] |
| - | [[Category: Gaboriaud | + | [[Category: Fontecilla-Camps JC]] |
| - | [[Category: Gruez | + | [[Category: Gaboriaud C]] |
| - | [[Category: Juanhuix | + | [[Category: Gruez A]] |
| - | [[Category: Lacroix | + | [[Category: Juanhuix J]] |
| - | [[Category: Pignol | + | [[Category: Lacroix M]] |
| - | [[Category: Verger | + | [[Category: Pignol D]] |
| - | + | [[Category: Verger D]] | |
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Current revision
Globular Head of the Complement System Protein C1q
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