1m0z: Difference between revisions

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-{{Seed}}
-[[Image:1m0z.png|left|200px]]
-<!--
+==Crystal Structure of the von Willebrand Factor Binding Domain of Glycoprotein Ib alpha==
-The line below this paragraph, containing "STRUCTURE_1m0z", creates the "Structure Box" on the page.
+<StructureSection load='1m0z' size='340' side='right'caption='[[1m0z]], [[Resolution|resolution]] 1.85&Aring;' scene=''>
-You may change the PDB parameter (which sets the PDB file loaded into the applet)
+== Structural highlights ==
-or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
+<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>
-or leave the SCENE parameter empty for the default display.
+</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>
-{{STRUCTURE_1m0z|  PDB=1m0z  |  SCENE=  }}
+</table>
+== 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>
-===Crystal Structure of the von Willebrand Factor Binding Domain of Glycoprotein Ib alpha===
+==See Also==
+*[[Platelet glycoprotein|Platelet glycoprotein]]
+== References ==
-<!--
+<references/>
-The line below this paragraph, {{ABSTRACT_PUBMED_12183630}}, adds the Publication Abstract to the page
+__TOC__
-(as it appears on PubMed at http://www.pubmed.gov), where 12183630 is the PubMed ID number.
+</StructureSection>
--->
-{{ABSTRACT_PUBMED_12183630}}
-==Disease==
-Known disease associated with this structure: Bernard-Soulier syndrome, type A OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=606672 606672]], von Willebrand disease, platelet-type OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=606672 606672]], Nonarteritic anterior ischemic optic neuropathy, susceptibility to OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=606672 606672]]
-==About this Structure==
-M0Z is a [[Single protein]] 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=1M0Z OCA].
-==Reference==
-Structures of glycoprotein Ibalpha and its complex with von Willebrand factor A1 domain., Huizinga EG, Tsuji S, Romijn RA, Schiphorst ME, de Groot PG, Sixma JJ, Gros P, Science. 2002 Aug 16;297(5584):1176-9. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/12183630 12183630]
 [[Category: Homo sapiens]]
-[[Category: Single protein]]
+[[Category: Large Structures]]
-[[Category: Groot, P G.de.]]
+[[Category: Gros P]]
-[[Category: Gros, P.]]
+[[Category: Huizinga EG]]
-[[Category: Huizinga, E G.]]
+[[Category: Romijn RAP]]
-[[Category: Romijn, R A.P.]]
+[[Category: Schiphorst ME]]
-[[Category: Schiphorst, M E.]]
+[[Category: Sixma JJ]]
-[[Category: Sixma, J J.]]
+[[Category: Tsuji S]]
-[[Category: Tsuji, S.]]
+[[Category: De Groot PG]]
-[[Category: Hemostasis]]
-[[Category: Leucine-rich repeat]]
-''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Wed Jul  2 22:58:41 2008''