期刊
BIOLOGICAL CHEMISTRY
卷 390, 期 5-6, 页码 391-400出版社
WALTER DE GRUYTER GMBH
DOI: 10.1515/BC.2009.057
关键词
Christmas factor; disorder-order transition; gatekeeper model; haemostasis; reversible activity modulation; zymogen
资金
- Austrian Science Fund FWF [P 23454] Funding Source: Medline
Blood haemostasis is accomplished by a complex network of coagulatory and fibrinolytic processes. These processes have to be delicately balanced, as clinically manifested by bleeding disorders, such as haemophilia A and B. These disorders are caused by defects in coagulation factor VIII and factor IX, respectively. Following a dual strategy, we emphasise on the one hand principles conserved in most coagulation enzymes, thus mirroring much of the underlying complexity in haemostasis; on the other hand, we identify enzymatic properties of the factor IXa-factor VIIIa system (Xase) that distinguish this proteolytic machine from other components of the coagulation system. While the exact mechanisms of its activity modulation remain baffling until today, superactive factor IX mutants significantly improve our current understanding and serve as a specific and testable model of Xase action.
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