2r2w

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[[Image:2r2w.png|left|200px]]
 
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{{STRUCTURE_2r2w| PDB=2r2w | SCENE= }}
{{STRUCTURE_2r2w| PDB=2r2w | SCENE= }}
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===Urokinase plasminogen activator B-chain-GPPE complex===
===Urokinase plasminogen activator B-chain-GPPE complex===
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{{ABSTRACT_PUBMED_17905583}}
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{{ABSTRACT_PUBMED_17905583}}
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==Disease==
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[[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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==Function==
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[[http://www.uniprot.org/uniprot/UROK_HUMAN UROK_HUMAN]] Specifically cleaves the zymogen plasminogen to form the active enzyme plasmin.
==About this Structure==
==About this Structure==
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==Reference==
==Reference==
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<ref group="xtra">PMID:017905583</ref><references group="xtra"/>
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<ref group="xtra">PMID:017905583</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: U-plasminogen activator]]
[[Category: U-plasminogen activator]]

Revision as of 00:35, 25 March 2013

Template:STRUCTURE 2r2w

Contents

Urokinase plasminogen activator B-chain-GPPE complex

Template:ABSTRACT PUBMED 17905583

Disease

[UROK_HUMAN] Defects in PLAU are the cause of Quebec platelet disorder (QPD) [MIM: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.[1]

Function

[UROK_HUMAN] Specifically cleaves the zymogen plasminogen to form the active enzyme plasmin.

About this Structure

2r2w is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

See Also

Reference

  • Zeslawska E, Sturzebecher J, Oleksyn BJ. Geometry of GPPE binding to picrate and to the urokinase type plasminogen activator. Bioorg Med Chem Lett. 2007 Nov 15;17(22):6212-5. Epub 2007 Sep 8. PMID:17905583 doi:10.1016/j.bmcl.2007.09.020
  1. ↑ Paterson AD, Rommens JM, Bharaj B, Blavignac J, Wong I, Diamandis M, Waye JS, Rivard GE, Hayward CP. Persons with Quebec platelet disorder have a tandem duplication of PLAU, the urokinase plasminogen activator gene. Blood. 2010 Feb 11;115(6):1264-6. doi: 10.1182/blood-2009-07-233965. Epub 2009, Dec 9. PMID:20007542 doi:10.1182/blood-2009-07-233965

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