1m0z: Difference between revisions

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-[[Image:1m0z.png|left|200px]]
-{{STRUCTURE_1m0z|  PDB=1m0z  |  SCENE=  }}
+==Crystal Structure of the von Willebrand Factor Binding Domain of Glycoprotein Ib alpha==
+<StructureSection load='1m0z' size='340' side='right'caption='[[1m0z]], [[Resolution|resolution]] 1.85&Aring;' scene=''>
-===Crystal Structure of the von Willebrand Factor Binding Domain of Glycoprotein Ib alpha===
+== Structural highlights ==
+<table><tr><td colspan='2'>[[1m0z]] 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=1M0Z OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1M0Z FirstGlance]. <br>
-{{ABSTRACT_PUBMED_12183630}}
+</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.85&#8491;</td></tr>
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=1m0z FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1m0z OCA], [https://pdbe.org/1m0z PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1m0z RCSB], [https://www.ebi.ac.uk/pdbsum/1m0z PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1m0z ProSAT]</span></td></tr>
-==About this Structure==
+</table>
-[[1m0z]] 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=1M0Z OCA].
+== Disease ==
+[https://www.uniprot.org/uniprot/GP1BA_HUMAN GP1BA_HUMAN] Genetic variations in GP1BA may be a cause of susceptibility to non-arteritic anterior ischemic optic neuropathy (NAION) [MIM:[https://omim.org/entry/258660 258660]. NAION is an ocular disease due to ischemic injury to the optic nerve. It usually affects the optic disk and leads to visual loss and optic disk swelling of a pallid nature. Visual loss is usually sudden, or over a few days at most and is usually permanent, with some recovery possibly occurring within the first weeks or months. Patients with small disks having smaller or non-existent cups have an anatomical predisposition for non-arteritic anterior ischemic optic neuropathy. As an ischemic episode evolves, the swelling compromises circulation, with a spiral of ischemia resulting in further neuronal damage.<ref>PMID:14711733</ref>   Defects in GP1BA are a cause of Bernard-Soulier syndrome (BSS) [MIM:[https://omim.org/entry/231200 231200]; also known as giant platelet disease (GPD). BSS patients have unusually large platelets and have a clinical bleeding tendency.<ref>PMID:1730088</ref> <ref>PMID:7690774</ref> <ref>PMID:7819107</ref> <ref>PMID:7873390</ref> <ref>PMID:9639514</ref> <ref>PMID:10089893</ref>   Defects in GP1BA are the cause of benign mediterranean macrothrombocytopenia (BMM) [MIM:[https://omim.org/entry/153670 153670]; also known as autosomal dominant benign Bernard-Soulier syndrome. BMM is characterized by mild or no clinical symptoms, normal platelet function, and normal megakaryocyte count.<ref>PMID:11222377</ref>   Defects in GP1BA are the cause of pseudo-von Willebrand disease (VWDP) [MIM:[https://omim.org/entry/177820 177820]. A bleeding disorder is caused by an increased affinity of GP-Ib for soluble vWF resulting in impaired hemostatic function due to the removal of vWF from the circulation.<ref>PMID:14521605</ref> <ref>PMID:2052556</ref> <ref>PMID:8486780</ref> <ref>PMID:8384898</ref>
+== Function ==
+[https://www.uniprot.org/uniprot/GP1BA_HUMAN GP1BA_HUMAN] GP-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to the A1 domain of vWF, which is already bound to the subendothelium.
+== Evolutionary Conservation ==
+[[Image:Consurf_key_small.gif|200px|right]]
+Check<jmol>
+  <jmolCheckbox>
+    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/m0/1m0z_consurf.spt"</scriptWhenChecked>
+    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
+    <text>to colour the structure by Evolutionary Conservation</text>
+  </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/main_output.php?pdb_ID=1m0z ConSurf].
+<div style="clear:both"></div>
 ==See Also==
-*[[Platelet-receptor glycoprotein Ib alpha|Platelet-receptor glycoprotein Ib alpha]]
+*[[Platelet glycoprotein|Platelet glycoprotein]]
+== References ==
-==Reference==
+<references/>
-<ref group="xtra">PMID:012183630</ref><references group="xtra"/>
+__TOC__
+</StructureSection>
 [[Category: Homo sapiens]]
-[[Category: Groot, P G.de.]]
+[[Category: Large Structures]]
-[[Category: Gros, P.]]
+[[Category: Gros P]]
-[[Category: Huizinga, E G.]]
+[[Category: Huizinga EG]]
-[[Category: Romijn, R A.P.]]
+[[Category: Romijn RAP]]
-[[Category: Schiphorst, M E.]]
+[[Category: Schiphorst ME]]
-[[Category: Sixma, J J.]]
+[[Category: Sixma JJ]]
-[[Category: Tsuji, S.]]
+[[Category: Tsuji S]]
-[[Category: Blood clotting]]
+[[Category: De Groot PG]]
-[[Category: Hemostasis]]
-[[Category: Leucine-rich repeat]]