2vnt

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== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[2vnt]] is a 6 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. This structure supersedes the now removed PDB entry [http://oca.weizmann.ac.il/oca-bin/send-pdb?obs=1&id=2jde 2jde]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2VNT OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2VNT FirstGlance]. <br>
<table><tr><td colspan='2'>[[2vnt]] is a 6 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. This structure supersedes the now removed PDB entry [http://oca.weizmann.ac.il/oca-bin/send-pdb?obs=1&id=2jde 2jde]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2VNT OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2VNT FirstGlance]. <br>
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</td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=QGG:1-({4-CHLORO-1-[(DIAMINOMETHYLIDENE)AMINO]ISOQUINOLIN-7-YL}SULFONYL)-D-PROLINE'>QGG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</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=QGG:1-({4-CHLORO-1-[(DIAMINOMETHYLIDENE)AMINO]ISOQUINOLIN-7-YL}SULFONYL)-D-PROLINE'>QGG</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
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<tr><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2viv|2viv]], [[1c5x|1c5x]], [[1gj9|1gj9]], [[2vio|2vio]], [[2vip|2vip]], [[1w0z|1w0z]], [[1fv9|1fv9]], [[1vja|1vja]], [[1gj8|1gj8]], [[1w11|1w11]], [[1sqo|1sqo]], [[1gjd|1gjd]], [[1w10|1w10]], [[1owk|1owk]], [[1w12|1w12]], [[1gi9|1gi9]], [[1o5a|1o5a]], [[1c5z|1c5z]], [[1owj|1owj]], [[1gi7|1gi7]], [[1o5b|1o5b]], [[1gjc|1gjc]], [[1sc8|1sc8]], [[1gja|1gja]], [[1o3p|1o3p]], [[1owe|1owe]], [[1sqa|1sqa]], [[1gjb|1gjb]], [[2viw|2viw]], [[1f92|1f92]], [[1owi|1owi]], [[1kdu|1kdu]], [[2vin|2vin]], [[1sqt|1sqt]], [[1f5l|1f5l]], [[1owd|1owd]], [[1ejn|1ejn]], [[2viq|2viq]], [[1lmw|1lmw]], [[1u6q|1u6q]], [[1gi8|1gi8]], [[1c5y|1c5y]], [[1w14|1w14]], [[1vj9|1vj9]], [[1w13|1w13]], [[1gj7|1gj7]], [[1c5w|1c5w]], [[1owh|1owh]], [[1o5c|1o5c]]</td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[2viv|2viv]], [[1c5x|1c5x]], [[1gj9|1gj9]], [[2vio|2vio]], [[2vip|2vip]], [[1w0z|1w0z]], [[1fv9|1fv9]], [[1vja|1vja]], [[1gj8|1gj8]], [[1w11|1w11]], [[1sqo|1sqo]], [[1gjd|1gjd]], [[1w10|1w10]], [[1owk|1owk]], [[1w12|1w12]], [[1gi9|1gi9]], [[1o5a|1o5a]], [[1c5z|1c5z]], [[1owj|1owj]], [[1gi7|1gi7]], [[1o5b|1o5b]], [[1gjc|1gjc]], [[1sc8|1sc8]], [[1gja|1gja]], [[1o3p|1o3p]], [[1owe|1owe]], [[1sqa|1sqa]], [[1gjb|1gjb]], [[2viw|2viw]], [[1f92|1f92]], [[1owi|1owi]], [[1kdu|1kdu]], [[2vin|2vin]], [[1sqt|1sqt]], [[1f5l|1f5l]], [[1owd|1owd]], [[1ejn|1ejn]], [[2viq|2viq]], [[1lmw|1lmw]], [[1u6q|1u6q]], [[1gi8|1gi8]], [[1c5y|1c5y]], [[1w14|1w14]], [[1vj9|1vj9]], [[1w13|1w13]], [[1gj7|1gj7]], [[1c5w|1c5w]], [[1owh|1owh]], [[1o5c|1o5c]]</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=2vnt FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2vnt OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2vnt RCSB], [http://www.ebi.ac.uk/pdbsum/2vnt 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=2vnt FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2vnt OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2vnt RCSB], [http://www.ebi.ac.uk/pdbsum/2vnt 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: Barber, C G.]]
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[[Category: Barber, C G]]
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[[Category: Brown, D G.]]
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[[Category: Brown, D G]]
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[[Category: Butt, R.]]
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[[Category: Butt, R]]
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[[Category: Collis, M G.]]
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[[Category: Collis, M G]]
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[[Category: Dickinson, R P.]]
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[[Category: Dickinson, R P]]
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[[Category: Fish, P V.]]
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[[Category: Fish, P V]]
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[[Category: Gara, M O.]]
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[[Category: Gara, M O]]
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[[Category: Henry, B T.]]
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[[Category: Henry, B T]]
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[[Category: Horne, V.]]
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[[Category: Horne, V]]
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[[Category: Huggins, J P.]]
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[[Category: Huggins, J P]]
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[[Category: King, E.]]
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[[Category: King, E]]
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[[Category: Mccleverty, D.]]
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[[Category: Mccleverty, D]]
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[[Category: Mcintosh, F.]]
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[[Category: Mcintosh, F]]
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[[Category: Phillips, C.]]
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[[Category: Phillips, C]]
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[[Category: Webster, R.]]
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[[Category: Webster, R]]
[[Category: Hydrolase]]
[[Category: Hydrolase]]
[[Category: Inhibitor complex]]
[[Category: Inhibitor complex]]
[[Category: Upa]]
[[Category: Upa]]

Revision as of 14:24, 19 January 2015

UROKINASE-TYPE PLASMINOGEN ACTIVATOR INHIBITOR COMPLEX WITH A 1-(7-SULPHOAMIDOISOQUINOLINYL)GUANIDINE

2vnt, resolution 2.20Å

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