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Comment
. 2002 Sep 3;99(18):11552-4.
doi: 10.1073/pnas.192448999. Epub 2002 Aug 23.

A new name in thrombosis, ADAMTS13

J Evan Sadler  1
Affiliations
Comment

A new name in thrombosis, ADAMTS13

J Evan Sadler. Proc Natl Acad Sci U S A. .
No abstract available

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Figures

Figure 1
Figure 1
Possible sites of ADAMTS13 action on VWF. VWF is released from endothelial cells as unusually large multimers that may diffuse into the circulation (A and B) or adhere to the endothelial cell surface (C). VWF also binds to connective tissue exposed at sites of vascular injury (D). Under conditions of high fluid shear stress, platelets can adhere to VWF in solution (B) or on surfaces (C and D) through the platelet glycoprotein Ib (GPIb) receptor. VWF also may recruit platelets to previously adhering platelets (E). ADAMTS13 cleaves a Tyr–Met bond in the A2 domain of the VWF subunit, severing the multimer. This reaction is slow for VWF in solution (A) but occurs rapidly when platelets adhere to VWF under high fluid shear conditions in suspension (B) or on surfaces (C–E), presumably as a consequence of conformational changes induced by tensile force on the VWF multimer. Failure of this mechanism appears to cause TTP.
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