1vj9: Difference between revisions
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{{STRUCTURE_1vj9| PDB=1vj9 | SCENE= }} | {{STRUCTURE_1vj9| PDB=1vj9 | SCENE= }} | ||
===Urokinase Plasminogen Activator B-Chain-JT464 Complex=== | |||
{{ABSTRACT_PUBMED_15150279}} | |||
=== | ==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. | |||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
<ref group="xtra">PMID:015150279</ref><references group="xtra"/> | <ref group="xtra">PMID:015150279</ref><references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: U-plasminogen activator]] | [[Category: U-plasminogen activator]] | ||
Revision as of 07:21, 25 March 2013
Urokinase Plasminogen Activator B-Chain-JT464 ComplexUrokinase Plasminogen Activator B-Chain-JT464 Complex
Template:ABSTRACT PUBMED 15150279
DiseaseDisease
[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]
FunctionFunction
[UROK_HUMAN] Specifically cleaves the zymogen plasminogen to form the active enzyme plasmin.
About this StructureAbout this Structure
1vj9 is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
See AlsoSee Also
ReferenceReference
- ↑ Schweinitz A, Steinmetzer T, Banke IJ, Arlt MJ, Sturzebecher A, Schuster O, Geissler A, Giersiefen H, Zeslawska E, Jacob U, Kruger A, Sturzebecher J. Design of novel and selective inhibitors of urokinase-type plasminogen activator with improved pharmacokinetic properties for use as antimetastatic agents. J Biol Chem. 2004 Aug 6;279(32):33613-22. Epub 2004 May 18. PMID:15150279 doi:10.1074/jbc.M314151200
- ↑ 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