3qn7
From Proteopedia
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{{STRUCTURE_3qn7| PDB=3qn7 | SCENE= }} | {{STRUCTURE_3qn7| PDB=3qn7 | SCENE= }} | ||
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===Potent and selective bicyclic peptide inhibitor (UK18) of human urokinase-type plasminogen activator(uPA)=== | ===Potent and selective bicyclic peptide inhibitor (UK18) of human urokinase-type plasminogen activator(uPA)=== | ||
| + | {{ABSTRACT_PUBMED_17692534}} | ||
| + | ==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> | ||
| - | + | ==Function== | |
| - | + | [[http://www.uniprot.org/uniprot/UROK_HUMAN UROK_HUMAN]] Specifically cleaves the zymogen plasminogen to form the active enzyme plasmin. | |
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==About this Structure== | ==About this Structure== | ||
[[3qn7]] is a 2 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=3QN7 OCA]. | [[3qn7]] is a 2 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=3QN7 OCA]. | ||
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| + | ==See Also== | ||
| + | *[[Urokinase|Urokinase]] | ||
==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:017692534</ref><ref group="xtra">PMID:008591045</ref><references group="xtra"/> | + | <ref group="xtra">PMID:017692534</ref><ref group="xtra">PMID:008591045</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: U-plasminogen activator]] | [[Category: U-plasminogen activator]] | ||
Revision as of 22:39, 24 March 2013
Contents |
Potent and selective bicyclic peptide inhibitor (UK18) of human urokinase-type plasminogen activator(uPA)
Template:ABSTRACT PUBMED 17692534
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
3qn7 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
See Also
Reference
- Zhao G, Yuan C, Wind T, Huang Z, Andreasen PA, Huang M. Structural basis of specificity of a peptidyl urokinase inhibitor, upain-1. J Struct Biol. 2007 Oct;160(1):1-10. Epub 2007 Jun 20. PMID:17692534 doi:10.1016/j.jsb.2007.06.003
- Spraggon G, Phillips C, Nowak UK, Ponting CP, Saunders D, Dobson CM, Stuart DI, Jones EY. The crystal structure of the catalytic domain of human urokinase-type plasminogen activator. Structure. 1995 Jul 15;3(7):681-91. PMID:8591045
- ↑ 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
