3rez

From Proteopedia

(Difference between revisions)

Current revision

glycoprotein GPIb variant

Structural highlights

3rez is a 4 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:	X-ray diffraction, Resolution 2.35Å
Ligands:	, , ,
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

GP1BB_HUMAN Bernard-Soulier syndrome;22q11.2 deletion syndrome;Fetal and neonatal alloimmune thrombocytopenia. The disease is caused by mutations affecting the gene represented in this entry.GPIX_HUMAN Bernard-Soulier syndrome. The disease is caused by variants affecting the gene represented in this entry.

Function

GP1BB_HUMAN Gp-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to von Willebrand factor, which is already bound to the subendothelium.GPIX_HUMAN The GPIb-V-IX complex functions as the vWF receptor and mediates vWF-dependent platelet adhesion to blood vessels. The adhesion of platelets to injured vascular surfaces in the arterial circulation is a critical initiating event in hemostasis. GP-IX may provide for membrane insertion and orientation of GP-Ib.

Publication Abstract from PubMed

Platelet GPIb-IX receptor complex has 3 subunits GPIbalpha, GPIbbeta, and GPIX, which assemble with a ratio of 1:2:1. Dysfunction in surface expression of the complex leads to Bernard-Soulier syndrome. We have crystallized the GPIbbeta ectodomain (GPIbbeta(E)) and determined the structure to show a single leucine-rich repeat with N- and C-terminal disulphide-bonded capping regions. The structure of a chimera of GPIbbeta(E) and 3 loops (a,b,c) taken from the GPIX ectodomain sequence was also determined. The chimera (GPIbbeta(Eabc)), but not GPIbbeta(E), forms a tetramer in the crystal, showing a quaternary interface between GPIbbeta and GPIX. Central to this interface is residue Tyr106 from GPIbbeta, which inserts into a pocket generated by 2 loops (b,c) from GPIX. Mutagenesis studies confirmed this interface as a valid representation of interactions between GPIbbeta and GPIX in the full-length complex. Eight GPIbbeta missense mutations identified from patients with Bernard-Soulier syndrome were examined for changes to GPIb-IX complex surface expression. Two mutations, A108P and P74R, were found to maintain normal secretion/folding of GPIbbeta(E) but were unable to support GPIX surface expression. The close structural proximity of these mutations to Tyr106 and the GPIbbeta(E) interface with GPIX indicates they disrupt the quaternary organization of the GPIb-IX complex.

Quaternary organization of GPIb-IX complex and insights into Bernard-Soulier syndrome revealed by the structures of GPIbbeta and a GPIbbeta/GPIX chimera.,McEwan PA, Yang W, Carr KH, Mo X, Zheng X, Li R, Emsley J Blood. 2011 Nov 10;118(19):5292-301. Epub 2011 Sep 8. PMID:21908432^[1]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

References

↑ McEwan PA, Yang W, Carr KH, Mo X, Zheng X, Li R, Emsley J. Quaternary organization of GPIb-IX complex and insights into Bernard-Soulier syndrome revealed by the structures of GPIbbeta and a GPIbbeta/GPIX chimera. Blood. 2011 Nov 10;118(19):5292-301. Epub 2011 Sep 8. PMID:21908432 doi:10.1182/blood-2011-05-356253

1 Structural highlights
2 Disease
3 Function
4 Publication Abstract from PubMed
5 References

Proteopedia Page Contributors and Editors (what is this?)

OCA

Retrieved from "http://52.214.119.220/wiki/index.php/3rez"

@@ Line 1: / Line 1: @@
-[[Image:3rez.jpg|left|200px]]
-<!--
+==glycoprotein GPIb variant==
-The line below this paragraph, containing "STRUCTURE_3rez", creates the "Structure Box" on the page.
+<StructureSection load='3rez' size='340' side='right'caption='[[3rez]], [[Resolution|resolution]] 2.35&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'>[[3rez]] is a 4 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=3REZ OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3REZ 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]] 2.35&#8491;</td></tr>
--->
+<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=FUL:BETA-L-FUCOSE'>FUL</scene>, <scene name='pdbligand=MAN:ALPHA-D-MANNOSE'>MAN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=PEG:DI(HYDROXYETHYL)ETHER'>PEG</scene></td></tr>
-{{STRUCTURE_3rez|  PDB=3rez  |  SCENE=  }}
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3rez FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3rez OCA], [https://pdbe.org/3rez PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3rez RCSB], [https://www.ebi.ac.uk/pdbsum/3rez PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3rez ProSAT]</span></td></tr>
+</table>
+== Disease ==
+[https://www.uniprot.org/uniprot/GP1BB_HUMAN GP1BB_HUMAN] Bernard-Soulier syndrome;22q11.2 deletion syndrome;Fetal and neonatal alloimmune thrombocytopenia. The disease is caused by mutations affecting the gene represented in this entry.[https://www.uniprot.org/uniprot/GPIX_HUMAN GPIX_HUMAN] Bernard-Soulier syndrome. The disease is caused by variants affecting the gene represented in this entry.
+== Function ==
+[https://www.uniprot.org/uniprot/GP1BB_HUMAN GP1BB_HUMAN] Gp-Ib, a surface membrane protein of platelets, participates in the formation of platelet plugs by binding to von Willebrand factor, which is already bound to the subendothelium.[https://www.uniprot.org/uniprot/GPIX_HUMAN GPIX_HUMAN] The GPIb-V-IX complex functions as the vWF receptor and mediates vWF-dependent platelet adhesion to blood vessels. The adhesion of platelets to injured vascular surfaces in the arterial circulation is a critical initiating event in hemostasis. GP-IX may provide for membrane insertion and orientation of GP-Ib.
+<div style="background-color:#fffaf0;">
+== Publication Abstract from PubMed ==
+Platelet GPIb-IX receptor complex has 3 subunits GPIbalpha, GPIbbeta, and GPIX, which assemble with a ratio of 1:2:1. Dysfunction in surface expression of the complex leads to Bernard-Soulier syndrome. We have crystallized the GPIbbeta ectodomain (GPIbbeta(E)) and determined the structure to show a single leucine-rich repeat with N- and C-terminal disulphide-bonded capping regions. The structure of a chimera of GPIbbeta(E) and 3 loops (a,b,c) taken from the GPIX ectodomain sequence was also determined. The chimera (GPIbbeta(Eabc)), but not GPIbbeta(E), forms a tetramer in the crystal, showing a quaternary interface between GPIbbeta and GPIX. Central to this interface is residue Tyr106 from GPIbbeta, which inserts into a pocket generated by 2 loops (b,c) from GPIX. Mutagenesis studies confirmed this interface as a valid representation of interactions between GPIbbeta and GPIX in the full-length complex. Eight GPIbbeta missense mutations identified from patients with Bernard-Soulier syndrome were examined for changes to GPIb-IX complex surface expression. Two mutations, A108P and P74R, were found to maintain normal secretion/folding of GPIbbeta(E) but were unable to support GPIX surface expression. The close structural proximity of these mutations to Tyr106 and the GPIbbeta(E) interface with GPIX indicates they disrupt the quaternary organization of the GPIb-IX complex.
-===glycoprotein GPIb variant===
+Quaternary organization of GPIb-IX complex and insights into Bernard-Soulier syndrome revealed by the structures of GPIbbeta and a GPIbbeta/GPIX chimera.,McEwan PA, Yang W, Carr KH, Mo X, Zheng X, Li R, Emsley J Blood. 2011 Nov 10;118(19):5292-301. Epub 2011 Sep 8. PMID:21908432<ref>PMID:21908432</ref>
+From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
-<!--
+</div>
-The line below this paragraph, {{ABSTRACT_PUBMED_21908432}}, adds the Publication Abstract to the page
+<div class="pdbe-citations 3rez" style="background-color:#fffaf0;"></div>
-(as it appears on PubMed at http://www.pubmed.gov), where 21908432 is the PubMed ID number.
+== References ==
--->
+<references/>
-{{ABSTRACT_PUBMED_21908432}}
+__TOC__
+</StructureSection>
-==About this Structure==
-[[3rez]] is a 4 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=3REZ OCA].
-==Reference==
-<ref group="xtra">PMID:021908432</ref><references group="xtra"/>
 [[Category: Homo sapiens]]
-[[Category: Carr, K H.]]
+[[Category: Large Structures]]
-[[Category: Emsley, J.]]
+[[Category: Carr KH]]
-[[Category: Li, R.]]
+[[Category: Emsley J]]
-[[Category: McEwan, P A.]]
+[[Category: Li R]]
-[[Category: Mo, X.]]
+[[Category: McEwan PA]]
-[[Category: Yang, W.]]
+[[Category: Mo X]]
-[[Category: Zheng, X.]]
+[[Category: Yang W]]
-[[Category: Cell adhesion]]
+[[Category: Zheng X]]
-[[Category: Glycoprotein]]
-[[Category: Gp1bb and gpix]]
-[[Category: Platelet surface receptor]]

3rez

From Proteopedia

Current revision

glycoprotein GPIb variant

Structural highlights

Disease

Function

Publication Abstract from PubMed

References

Contents

Proteopedia Page Contributors and Editors (what is this?)

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