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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。
Thromb Haemost. 2013 Jul;110(1):202-4. doi: 10.1160/TH13-01-0053. Epub 2013 May 2.
2
[Increased susceptibility of recombinant type 2A von Willebrand factor mutant A1500E to proteolysis by ADAMTS13].[重组2A型血管性血友病因子突变体A1500E对ADAMTS13蛋白水解的敏感性增加]
Zhonghua Xue Ye Xue Za Zhi. 2012 Mar;33(3):169-72.
3
Normal cleavage of von Willebrand factor by ADAMTS-13 in the absence of factor VIII in patients with severe hemophilia A.在重度A型血友病患者中,缺乏凝血因子VIII时,ADAMTS - 13对血管性血友病因子的正常裂解作用。
J Thromb Haemost. 2013 Sep;11(9):1769-72. doi: 10.1111/jth.12299.
4
A cluster of mutations in the D3 domain of von Willebrand factor correlates with a distinct subgroup of von Willebrand disease: type 2A/IIE.血管性血友病因子 D3 结构域内的一簇突变与血管性血友病的一个独特亚群相关:2A/IIE 型。
Blood. 2010 Jun 10;115(23):4894-901. doi: 10.1182/blood-2009-07-226324. Epub 2010 Mar 29.
5
Relevance of chloride binding to von Willebrand factor in type 2B von Willebrand disease patients.2B型血管性血友病患者中氯离子与血管性血友病因子结合的相关性
J Thromb Haemost. 2010 Feb;8(2):416-8. doi: 10.1111/j.1538-7836.2009.03706.x. Epub 2009 Nov 26.
6
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.
7
Structural basis of type 2A von Willebrand disease investigated by molecular dynamics simulations and experiments.通过分子动力学模拟和实验研究 2A 型血管性血友病的结构基础。
PLoS One. 2012;7(10):e45207. doi: 10.1371/journal.pone.0045207. Epub 2012 Oct 23.
8
Intersection of mechanisms of type 2A VWD through defects in VWF multimerization, secretion, ADAMTS-13 susceptibility, and regulated storage.2A 型血管性血友病通过 VWF 多聚体化、分泌、ADAMTS-13 易感性和调控储存缺陷的机制交汇。
Blood. 2012 May 10;119(19):4543-53. doi: 10.1182/blood-2011-06-360875. Epub 2012 Mar 19.
9
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.
10
Modeling ADAMTS13-von Willebrand factor interaction: Implications for oxidative stress-related cardiovascular diseases and type 2A von Willebrand disease.模拟 ADAMTS13-血管性血友病因子相互作用:与氧化应激相关的心血管疾病和 2A 型血管性血友病的关系。
Biophys Chem. 2012 Jan;160(1):1-11. doi: 10.1016/j.bpc.2011.07.007. Epub 2011 Aug 3.

引用本文的文献

1
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。
J Thromb Haemost. 2020 Sep;18(9):2169-2176. doi: 10.1111/jth.14962. Epub 2020 Jul 23.

本文引用的文献

1
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.
4
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.
8
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
J Thromb Haemost. 2006 Oct;4(10):2103-14. doi: 10.1111/j.1538-7836.2006.02146.x. Epub 2006 Aug 2.
9
Recombinant expression of mutations causing von Willebrand disease type Normandy: characterization of a combined defect of factor VIII binding and multimerization.导致诺曼底型血管性血友病的突变的重组表达:VIII因子结合与多聚化联合缺陷的特征分析
Thromb Haemost. 2004 Jul;92(1):36-41. doi: 10.1160/TH04-02-0084.
10
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。
Blood. 2003 Jan 1;101(1):151-6. doi: 10.1182/blood-2002-04-1046. Epub 2002 Aug 8.

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.

DOI:10.1160/TH13-01-0053
PMID:23636243
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3813456/
Abstract
摘要