期刊
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
卷 16, 期 1, 页码 6-18出版社
WILEY
DOI: 10.1111/jth.13898
关键词
ADAMTS-13; conformational activation; hemostasis; TTP; VWF
资金
- British Heart Foundation [PG/12/55/29740, FS/14/21/30733, PG/14/87/31181]
- British Heart Foundation [FS/14/21/30733, PG/12/55/29740, PG/14/87/31181] Funding Source: researchfish
von Willebrand factor (VWF) is a key player in hemostasis, acting as a carrier for factor VIII and capturing platelets at sites of vascular damage. To capture platelets, it must undergo conformational changes, both within its A1 domain and at the macromolecular level through A2 domain unfolding. Its size and this function are regulated by the metalloproteinase ADAMTS-13. Recently, it has been shown that ADAMTS-13 undergoes a conformational change upon interaction with VWF, and that this enhances its activity towards its substrate. This review summarizes recent work on these conformational transitions, describing how they are controlled. It points to their importance in hemostasis, bleeding disorders, and the developing field of therapeutic application of ADAMTS-13 as an antithrombotic agent in obstructive microvascular thrombosis and in cardiovascular disease.
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