1sqo

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== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[1sqo]] 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=1SQO OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1SQO FirstGlance]. <br>
<table><tr><td colspan='2'>[[1sqo]] 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=1SQO OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1SQO FirstGlance]. <br>
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</td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=UI2:8-(PYRIMIDIN-2-YLAMINO)NAPHTHALENE-2-CARBOXIMIDAMIDE'>UI2</scene><br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=UI2:8-(PYRIMIDIN-2-YLAMINO)NAPHTHALENE-2-CARBOXIMIDAMIDE'>UI2</scene></td></tr>
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<tr><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1owd|1owd]], [[1owe|1owe]], [[1owh|1owh]], [[1owi|1owi]], [[1owj|1owj]], [[1owk|1owk]], [[1sqa|1sqa]], [[1sqt|1sqt]]</td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1owd|1owd]], [[1owe|1owe]], [[1owh|1owh]], [[1owi|1owi]], [[1owj|1owj]], [[1owk|1owk]], [[1sqa|1sqa]], [[1sqt|1sqt]]</td></tr>
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<tr><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">PLAU ([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='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">PLAU ([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><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/U-plasminogen_activator U-plasminogen activator], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.21.73 3.4.21.73] </span></td></tr>
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/U-plasminogen_activator U-plasminogen activator], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.4.21.73 3.4.21.73] </span></td></tr>
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<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1sqo FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1sqo OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1sqo RCSB], [http://www.ebi.ac.uk/pdbsum/1sqo PDBsum]</span></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=1sqo FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1sqo OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1sqo RCSB], [http://www.ebi.ac.uk/pdbsum/1sqo PDBsum]</span></td></tr>
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<table>
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</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/UROK_HUMAN UROK_HUMAN]] Defects in PLAU are the cause of Quebec platelet disorder (QPD) [MIM:[http://omim.org/entry/601709 601709]]. QPD is an autosomal dominant bleeding disorder due to a gain-of-function defect in fibrinolysis. Although affected individuals do not exhibit systemic fibrinolysis, they show delayed onset bleeding after challenge, such as surgery. The hallmark of the disorder is markedly increased PLAU levels within platelets, which causes intraplatelet plasmin generation and secondary degradation of alpha-granule proteins.<ref>PMID:20007542</ref>
[[http://www.uniprot.org/uniprot/UROK_HUMAN UROK_HUMAN]] Defects in PLAU are the cause of Quebec platelet disorder (QPD) [MIM:[http://omim.org/entry/601709 601709]]. QPD is an autosomal dominant bleeding disorder due to a gain-of-function defect in fibrinolysis. Although affected individuals do not exhibit systemic fibrinolysis, they show delayed onset bleeding after challenge, such as surgery. The hallmark of the disorder is markedly increased PLAU levels within platelets, which causes intraplatelet plasmin generation and secondary degradation of alpha-granule proteins.<ref>PMID:20007542</ref>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: U-plasminogen activator]]
[[Category: U-plasminogen activator]]
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[[Category: Geyer, A.]]
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[[Category: Geyer, A]]
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[[Category: Giranda, V L.]]
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[[Category: Giranda, V L]]
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[[Category: Klinghofer, V.]]
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[[Category: Klinghofer, V]]
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[[Category: Mantei, R.]]
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[[Category: Mantei, R]]
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[[Category: McClellan, W J.]]
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[[Category: McClellan, W J]]
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[[Category: Nienaber, V.]]
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[[Category: Nienaber, V]]
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[[Category: Rockway, T W.]]
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[[Category: Rockway, T W]]
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[[Category: Stewart, K.]]
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[[Category: Stewart, K]]
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[[Category: Weitzberg, M.]]
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[[Category: Weitzberg, M]]
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[[Category: Wendt, M D.]]
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[[Category: Wendt, M D]]
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[[Category: Zhao, X.]]
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[[Category: Zhao, X]]
[[Category: Egf-like domain]]
[[Category: Egf-like domain]]
[[Category: Glycoprotein]]
[[Category: Glycoprotein]]

Revision as of 08:20, 6 January 2015

Substituted 2-Naphthamidine Inhibitors of Urokinase

1sqo, resolution 1.84Å

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