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1nzi

From Proteopedia

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{{STRUCTURE_1nzi| PDB=1nzi | SCENE= }}
 
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===Crystal Structure of the CUB1-EGF Interaction Domain of Complement Protease C1s===
 
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{{ABSTRACT_PUBMED_12788922}}
 
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==Disease==
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==Crystal Structure of the CUB1-EGF Interaction Domain of Complement Protease C1s==
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[[http://www.uniprot.org/uniprot/C1S_HUMAN C1S_HUMAN]] Defects in C1S are the cause of complement component C1s deficiency (C1SD) [MIM:[http://omim.org/entry/613783 613783]]. 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.
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<StructureSection load='1nzi' size='340' side='right'caption='[[1nzi]], [[Resolution|resolution]] 1.50&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[1nzi]] is a 2 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=1NZI OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1NZI FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/C1S_HUMAN C1S_HUMAN]] C1s B chain is a serine protease that combines with C1q and C1r to form C1, the first component of the classical pathway of the complement system. C1r activates C1s so that it can, in turn, activate C2 and C4.
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.5&#8491;</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene></td></tr>
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==About this Structure==
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1nzi FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1nzi OCA], [https://pdbe.org/1nzi PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1nzi RCSB], [https://www.ebi.ac.uk/pdbsum/1nzi PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1nzi ProSAT]</span></td></tr>
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[[1nzi]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1NZI OCA].
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</table>
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== Disease ==
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==Reference==
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[https://www.uniprot.org/uniprot/C1S_HUMAN C1S_HUMAN] Defects in C1S are the cause of complement component C1s deficiency (C1SD) [MIM:[https://omim.org/entry/613783 613783]. 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.
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<ref group="xtra">PMID:012788922</ref><references group="xtra"/><references/>
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== Function ==
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[[Category: Complement subcomponent C1s]]
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[https://www.uniprot.org/uniprot/C1S_HUMAN C1S_HUMAN] C1s B chain is a serine protease that combines with C1q and C1r to form C1, the first component of the classical pathway of the complement system. C1r activates C1s so that it can, in turn, activate C2 and C4.
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/nz/1nzi_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</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=1nzi ConSurf].
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<div style="clear:both"></div>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Arlaud, G J.]]
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[[Category: Large Structures]]
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[[Category: Fontecilla-Camps, J C.]]
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[[Category: Arlaud GJ]]
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[[Category: Gaboriaud, C.]]
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[[Category: Fontecilla-Camps JC]]
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[[Category: Gregory, L A.]]
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[[Category: Gaboriaud C]]
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[[Category: Thielens, N M.]]
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[[Category: Gregory LA]]
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[[Category: Complement]]
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[[Category: Thielens NM]]
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[[Category: Cub]]
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[[Category: Egf]]
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[[Category: Hydrolase]]
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[[Category: Innate immunity]]
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[[Category: Modular structure]]
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Current revision

Crystal Structure of the CUB1-EGF Interaction Domain of Complement Protease C1s

PDB ID 1nzi

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