2oay

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{{Seed}}
 
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[[Image:2oay.png|left|200px]]
 
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==Crystal structure of latent human C1-inhibitor==
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The line below this paragraph, containing "STRUCTURE_2oay", creates the "Structure Box" on the page.
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<StructureSection load='2oay' size='340' side='right'caption='[[2oay]], [[Resolution|resolution]] 2.35&Aring;' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[2oay]] is a 1 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=2OAY OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2OAY FirstGlance]. <br>
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or leave the SCENE parameter empty for the default display.
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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]] 2.35&#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=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr>
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{{STRUCTURE_2oay| PDB=2oay | SCENE= }}
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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=2oay FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2oay OCA], [https://pdbe.org/2oay PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2oay RCSB], [https://www.ebi.ac.uk/pdbsum/2oay PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2oay ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/IC1_HUMAN IC1_HUMAN] Defects in SERPING1 are the cause of hereditary angioedema (HAE) [MIM:[https://omim.org/entry/106100 106100]; also called hereditary angioneurotic edema (HANE). HAE is an autosomal dominant disorder characterized by episodic local subcutaneous edema and submucosal edema involving the upper respiratory and gastrointestinal tracts. HAE due to C1 esterase inhibitor deficiency is comprised of two clinically indistinguishable forms. In HAE type 1, representing 85% of patients, serum levels of C1 esterase inhibitor are less than 35% of normal. In HAE type 2, the levels are normal or elevated, but the protein is non-functional.<ref>PMID:12773530</ref> <ref>PMID:7814636</ref> <ref>PMID:3178731</ref> <ref>PMID:2365061</ref> <ref>PMID:2296585</ref> <ref>PMID:2118657</ref> [:]<ref>PMID:1451784</ref> <ref>PMID:1363816</ref> <ref>PMID:8172583</ref> <ref>PMID:7883978</ref> <ref>PMID:8529136</ref> <ref>PMID:8755917</ref> <ref>PMID:14635117</ref> <ref>PMID:16409206</ref> <ref>PMID:22994404</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/IC1_HUMAN IC1_HUMAN] Activation of the C1 complex is under control of the C1-inhibitor. It forms a proteolytically inactive stoichiometric complex with the C1r or C1s proteases. May play a potentially crucial role in regulating important physiological pathways including complement activation, blood coagulation, fibrinolysis and the generation of kinins. Very efficient inhibitor of FXIIa. Inhibits chymotrypsin and kallikrein.<ref>PMID:8495195</ref>
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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/oa/2oay_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview03.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=2oay ConSurf].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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C1 inhibitor, a member of the serpin family, is a major down-regulator of inflammatory processes in blood. Genetic deficiency of C1 inhibitor results in hereditary angioedema, a dominantly inheritable, potentially lethal disease. Here we report the first crystal structure of the serpin domain of human C1 inhibitor, representing a previously unreported latent form, which explains functional consequences of several naturally occurring mutations, two of which are discussed in detail. The presented structure displays a novel conformation with a seven-stranded beta-sheet A. The unique conformation of the C-terminal six residues suggests its potential role as a barrier in the active-latent transition. On the basis of surface charge pattern, heparin affinity measurements, and docking of a heparin disaccharide, a heparin binding site is proposed in the contact area of the serpin-proteinase encounter complex. We show how polyanions change the activity of the C1 inhibitor by a novel "sandwich" mechanism, explaining earlier reaction kinetic and mutagenesis studies. These results may help to improve therapeutic C1 inhibitor preparations used in the treatment of hereditary angioedema, organ transplant rejection, and heart attack.
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===Crystal structure of latent human C1-inhibitor===
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C1 inhibitor serpin domain structure reveals the likely mechanism of heparin potentiation and conformational disease.,Beinrohr L, Harmat V, Dobo J, Lorincz Z, Gal P, Zavodszky P J Biol Chem. 2007 Jul 20;282(29):21100-9. Epub 2007 May 8. PMID:17488724<ref>PMID:17488724</ref>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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The line below this paragraph, {{ABSTRACT_PUBMED_17488724}}, adds the Publication Abstract to the page
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<div class="pdbe-citations 2oay" style="background-color:#fffaf0;"></div>
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(as it appears on PubMed at http://www.pubmed.gov), where 17488724 is the PubMed ID number.
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== References ==
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<references/>
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{{ABSTRACT_PUBMED_17488724}}
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__TOC__
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</StructureSection>
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==About this Structure==
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2OAY is a 1 chain structure of 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=2OAY OCA].
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==Reference==
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<ref group="xtra">PMID:17488724</ref><references group="xtra"/>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Beinrohr, L.]]
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[[Category: Large Structures]]
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[[Category: Dobo, J.]]
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[[Category: Beinrohr L]]
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[[Category: Gal, P.]]
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[[Category: Dobo J]]
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[[Category: Harmat, V.]]
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[[Category: Gal P]]
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[[Category: Latent serpin]]
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[[Category: Harmat V]]
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[[Category: Rcl insertion]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Feb 17 10:00:37 2009''
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Current revision

Crystal structure of latent human C1-inhibitor

PDB ID 2oay

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