4gly

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'''Unreleased structure'''
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{{STRUCTURE_4gly| PDB=4gly | SCENE= }}
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===Human urokinase-type plasminogen activator uPA in complex with the two-disulfide bridge peptide UK504===
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{{ABSTRACT_PUBMED_23560397}}
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The entry 4gly is ON HOLD until Paper Publication
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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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Authors: Buth, S.A., Leiman, P.G., Chen, S., Heinis, C.
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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.
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Description: Human urokinase-type plasminogen activator uPA in complex with the two-disulfide bridge peptide UK504
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==About this Structure==
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[[4gly]] 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=4GLY OCA].
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==Reference==
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<references group="xtra"/><references/>
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[[Category: Homo sapiens]]
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[[Category: U-plasminogen activator]]
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[[Category: Buth, S A.]]
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[[Category: Chen, S.]]
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[[Category: Heinis, C.]]
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[[Category: Leiman, P G.]]
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[[Category: Bicyclic peptide inhibitor]]
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[[Category: Chymotrypsin fold]]
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[[Category: Hydrolase-hydrolase inhibitor complex]]
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[[Category: Serine protease]]
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[[Category: Urokinase-type plasminogen activator]]

Revision as of 07:23, 16 May 2013

Template:STRUCTURE 4gly

Contents

Human urokinase-type plasminogen activator uPA in complex with the two-disulfide bridge peptide UK504

Template:ABSTRACT PUBMED 23560397

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

4gly is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  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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OCA

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