Compromised shear-dependent cleavage of type 2N von Willebrand factor variants by ADAMTS13 in the presence of factor VIII.
作者信息
Skipwith Christopher G, Haberichter Sandra L, Gehrand Ashley, Zheng X Long
出版信息
Thromb Haemost. 2013 Jul;110(1):202-4. doi: 10.1160/TH13-01-0053. Epub 2013 May 2.
Abstract
摘要
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Compromised shear-dependent cleavage of type 2N von Willebrand factor variants by ADAMTS13 in the presence of factor VIII.在因子VIII存在的情况下，ADAMTS13对2N型血管性血友病因子变体的剪切依赖性裂解受损。
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Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress.VIII 因子和血小板在切应力下通过 ADAMTS13 协同加速 von Willebrand 因子的裂解。
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Factor VIII binding affects the mechanical unraveling of the A2 domain of von Willebrand factor.VIII 因子结合影响 von Willebrand 因子 A2 结构域的机械展开。
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Light chain of factor VIII is sufficient for accelerating cleavage of von Willebrand factor by ADAMTS13 metalloprotease.VIII 因子轻链足以加速 ADAMTS13 金属蛋白酶对血管性血友病因子的裂解。
J Biol Chem. 2012 Sep 21;287(39):32459-66. doi: 10.1074/jbc.M112.390690. Epub 2012 Aug 1.
2
von Willebrand factor (VWF) propeptide binding to VWF D'D3 domain attenuates platelet activation and adhesion.血管性血友病因子前肽与血管性血友病因子 D'D3 结构域结合可减弱血小板的激活和黏附。
Blood. 2012 May 17;119(20):4769-78. doi: 10.1182/blood-2011-10-387548. Epub 2012 Mar 27.
3
Validation of the first commercial ELISA for type 2N von Willebrand's disease diagnosis.验证首个用于 2N 型血管性血友病诊断的商业 ELISA。
Haemophilia. 2011 Nov;17(6):944-51. doi: 10.1111/j.1365-2516.2011.02499.x. Epub 2011 Mar 3.
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Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress.VIII 因子和血小板在切应力下通过 ADAMTS13 协同加速 von Willebrand 因子的裂解。
J Biol Chem. 2010 Sep 10;285(37):28596-603. doi: 10.1074/jbc.M110.131227. Epub 2010 Jul 6.
5
The genetic basis of von Willebrand disease.血管性血友病的遗传学基础。
Blood Rev. 2010 May;24(3):123-34. doi: 10.1016/j.blre.2010.03.003. Epub 2010 Apr 20.
6
Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13.凝血因子VIII可加速ADAMTS13对血管性血友病因子的蛋白水解切割。
Proc Natl Acad Sci U S A. 2008 May 27;105(21):7416-21. doi: 10.1073/pnas.0801735105. Epub 2008 May 20.
7
Exosite interactions contribute to tension-induced cleavage of von Willebrand factor by the antithrombotic ADAMTS13 metalloprotease.外位点相互作用有助于抗血栓性ADAMTS13金属蛋白酶对血管性血友病因子的张力诱导切割。
Proc Natl Acad Sci U S A. 2006 Dec 12;103(50):19099-104. doi: 10.1073/pnas.0607264104. Epub 2006 Dec 4.
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Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor.血管性血友病的病理生理学与分类更新：血管性血友病因子小组委员会报告
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An Arg760Cys mutation in the consensus sequence of the von Willebrand factor propeptide cleavage site is responsible for a new von Willebrand disease variant.血管性血友病因子前肽裂解位点共有序列中的Arg760Cys突变导致了一种新型血管性血友病疾病变体。
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