3m61
From Proteopedia
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- | [[Image:3m61.png|left|200px]] | ||
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{{STRUCTURE_3m61| PDB=3m61 | SCENE= }} | {{STRUCTURE_3m61| PDB=3m61 | SCENE= }} | ||
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===Crystal structure of complex of urokinase and a upain-1 variant(W3A) in pH4.6 condition=== | ===Crystal structure of complex of urokinase and a upain-1 variant(W3A) in pH4.6 condition=== | ||
+ | ==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> | ||
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+ | ==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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==See Also== | ==See Also== | ||
*[[Urokinase|Urokinase]] | *[[Urokinase|Urokinase]] | ||
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+ | ==Reference== | ||
+ | <references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: U-plasminogen activator]] | [[Category: U-plasminogen activator]] |
Revision as of 12:08, 24 March 2013
Contents |
Crystal structure of complex of urokinase and a upain-1 variant(W3A) in pH4.6 condition
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
3m61 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
See Also
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
Categories: Homo sapiens | U-plasminogen activator | Andreasen, P A. | Chen, L. | Huang, M. | Jiang, L. | Meehan, E J. | Wind, T. | Yuan, C. | Blood coagulation | Disulfide bond | Egf-like domain | Fibrinolysis | Glycoprotein | Hydrolase | Hydrolase-hydrolase inhibitor complex | Kringle | Phosphoprotein | Plasminogen activation | Protease | Protein-ligand complex | Secreted | Serine protease | Serine protease-peptide complex | Zymogen