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3ghm

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{{STRUCTURE_3ghm| PDB=3ghm | SCENE= }}
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==Crystal structure of the exosite-containing fragment of human ADAMTS13 (form-1)==
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===Crystal structure of the exosite-containing fragment of human ADAMTS13 (form-1)===
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<StructureSection load='3ghm' size='340' side='right' caption='[[3ghm]], [[Resolution|resolution]] 2.60&Aring;' scene=''>
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{{ABSTRACT_PUBMED_19574655}}
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== Structural highlights ==
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<table><tr><td colspan='2'>[[3ghm]] is a 1 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=3GHM OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3GHM FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BGC:BETA-D-GLUCOSE'>BGC</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=FUC:ALPHA-L-FUCOSE'>FUC</scene></td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[3ghn|3ghn]]</td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ADAMTS13 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3ghm FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3ghm OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3ghm RCSB], [http://www.ebi.ac.uk/pdbsum/3ghm PDBsum]</span></td></tr>
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</table>
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== Disease ==
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[[http://www.uniprot.org/uniprot/ATS13_HUMAN ATS13_HUMAN]] Defects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP) [MIM:[http://omim.org/entry/274150 274150]]; also known as Upshaw-Schulman syndrome (USS). A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever.<ref>PMID:11586351</ref> <ref>PMID:12181489</ref> <ref>PMID:12393505</ref> <ref>PMID:12614216</ref> <ref>PMID:12753286</ref> <ref>PMID:14512317</ref> <ref>PMID:14563640</ref> <ref>PMID:15126318</ref> <ref>PMID:15009458</ref> <ref>PMID:15327386</ref> <ref>PMID:16160007</ref> <ref>PMID:16453338</ref> <ref>PMID:16796708</ref> <ref>PMID:16449289</ref> <ref>PMID:16807643</ref> <ref>PMID:17003922</ref> <ref>PMID:18443791</ref> <ref>PMID:19055667</ref> <ref>PMID:19116307</ref> <ref>PMID:22075512</ref>
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== Function ==
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[[http://www.uniprot.org/uniprot/ATS13_HUMAN ATS13_HUMAN]] Cleaves the vWF multimers in plasma into smaller forms.
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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/gh/3ghm_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/chain_selection.php?pdb_ID=2ata 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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ADAMTS13 is a reprolysin-type metalloproteinase belonging to the ADAMTS (a disintegrin and metalloproteinase with thrombospondin type 1 motif) family. It specifically cleaves plasma von Willebrand factor (VWF) and regulates platelet adhesion and aggregation. ADAMTS13 is a multi-domain enzyme. In addition to the N-terminal metalloproteinase domain, the ancillary domains, including a disintegrin-like domain, a thrombospondin-1 type 1 repeat, a Cys-rich domain and a spacer domain, are required for VWF recognition and cleavage. In the present study, a fragment of the ADAMTS13 ancillary domains (ADAMTS13-DTCS; residues 287-685) was expressed using CHO Lec cells, purified and crystallized. Diffraction data sets were collected using the SPring-8 beamline. Two ADAMTS13-DTCS crystals with distinct unit-cell parameters generated data sets to 2.6 and 2.8 A resolution, respectively.
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==Disease==
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Production, crystallization and preliminary crystallographic analysis of an exosite-containing fragment of human von Willebrand factor-cleaving proteinase ADAMTS13.,Akiyama M, Takeda S, Kokame K, Takagi J, Miyata T Acta Crystallogr Sect F Struct Biol Cryst Commun. 2009 Jul 1;65(Pt, 7):739-42. Epub 2009 Jun 30. PMID:19574655<ref>PMID:19574655</ref>
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[[http://www.uniprot.org/uniprot/ATS13_HUMAN ATS13_HUMAN]] Defects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP) [MIM:[http://omim.org/entry/274150 274150]]; also known as Upshaw-Schulman syndrome (USS). A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever.<ref>PMID:11586351</ref><ref>PMID:12181489</ref><ref>PMID:12393505</ref><ref>PMID:12614216</ref><ref>PMID:12753286</ref><ref>PMID:14512317</ref><ref>PMID:14563640</ref><ref>PMID:15126318</ref><ref>PMID:15009458</ref><ref>PMID:15327386</ref><ref>PMID:16160007</ref><ref>PMID:16453338</ref><ref>PMID:16796708</ref><ref>PMID:16449289</ref><ref>PMID:16807643</ref><ref>PMID:17003922</ref><ref>PMID:18443791</ref><ref>PMID:19055667</ref><ref>PMID:19116307</ref><ref>PMID:22075512</ref>
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==Function==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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[[http://www.uniprot.org/uniprot/ATS13_HUMAN ATS13_HUMAN]] Cleaves the vWF multimers in plasma into smaller forms.
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</div>
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==About this Structure==
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[[3ghm]] is a 1 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=3GHM OCA].
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==See Also==
==See Also==
*[[A Disintegrin And Metalloproteinase|A Disintegrin And Metalloproteinase]]
*[[A Disintegrin And Metalloproteinase|A Disintegrin And Metalloproteinase]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:019574655</ref><ref group="xtra">PMID:019880749</ref><references group="xtra"/><references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Akiyama, M.]]
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[[Category: Akiyama, M]]
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[[Category: Kokame, K.]]
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[[Category: Kokame, K]]
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[[Category: Miyata, T.]]
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[[Category: Miyata, T]]
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[[Category: Takagi, J.]]
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[[Category: Takagi, J]]
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[[Category: Takeda, S.]]
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[[Category: Takeda, S]]
[[Category: Beta sandwich]]
[[Category: Beta sandwich]]
[[Category: Hydrolase]]
[[Category: Hydrolase]]
[[Category: Thrombospondin type-1 motif]]
[[Category: Thrombospondin type-1 motif]]

Revision as of 16:19, 18 December 2014

Crystal structure of the exosite-containing fragment of human ADAMTS13 (form-1)

3ghm, resolution 2.60Å

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