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4jnl
From Proteopedia
(Difference between revisions)
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| - | + | {{STRUCTURE_4jnl| PDB=4jnl | SCENE= }} | |
| + | ===Crystal Structure of uPA in complex with its inhibitor 4-bromobenzylamine at pH 7.4=== | ||
| - | + | ==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== | |
| + | [[4jnl]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4JNL OCA]. | ||
| + | |||
| + | ==Reference== | ||
| + | <references group="xtra"/><references/> | ||
| + | [[Category: U-plasminogen activator]] | ||
| + | [[Category: Huang, M.]] | ||
| + | [[Category: Jiang, L.]] | ||
| + | [[Category: Yuan, C.]] | ||
| + | [[Category: Zhang, X.]] | ||
| + | [[Category: Zhao, B.]] | ||
| + | [[Category: Halogen]] | ||
| + | [[Category: Hydrolase-hydrolase inhibitor complex]] | ||
| + | [[Category: Upa]] | ||
Revision as of 09:31, 19 March 2014
Contents |
Crystal Structure of uPA in complex with its inhibitor 4-bromobenzylamine at pH 7.4
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
4jnl is a 1 chain structure. Full crystallographic information is available from OCA.
Reference
- ↑ 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
