3ox7
The crystal structure of uPA complex with peptide inhibitor MH027 at pH4.6The crystal structure of uPA complex with peptide inhibitor MH027 at pH4.6
Template:ABSTRACT PUBMED 21802428
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
3ox7 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
ReferenceReference
- ↑ Jiang L, Svane AS, Sorensen HP, Jensen JK, Hosseini M, Chen Z, Weydert C, Nielsen JT, Christensen A, Yuan C, Jensen KJ, Nielsen NC, Malmendal A, Huang M, Andreasen PA. The binding mechanism of a peptidic cyclic serine protease inhibitor. J Mol Biol. 2011 Sep 16;412(2):235-50. Epub 2011 Jul 23. PMID:21802428 doi:10.1016/j.jmb.2011.07.028
- ↑ 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