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Cited by 444

The DOI matches its Crossref record; 67 of the 67 references it cites were found in a registry or an index.

von Willebrand factor, Jedi knight of the bloodstream

Open access
Springer, Timothy A.·2014·Blood

Abstract

When blood vessels are cut, the forces in the bloodstream increase and change character. The dark side of these forces causes hemorrhage and death. However, von Willebrand factor (VWF), with help from our circulatory system and platelets, harnesses the same forces to form a hemostatic plug. Force and VWF function are so closely intertwined that, like members of the Jedi Order in the movie Star Wars who learn to use “the Force” to do good, VWF may be considered the Jedi knight of the bloodstream. The long length of VWF enables responsiveness to flow. The shape of VWF is predicted to alter from irregularly coiled to extended thread-like in the transition from shear to elongational flow at sites of hemostasis and thrombosis. Elongational force propagated through the length of VWF in its thread-like shape exposes its monomers for multimeric binding to platelets and subendothelium and likely also increases affinity of the A1 domain for platelets. Specialized domains concatenate and compact VWF during biosynthesis. A2 domain unfolding by hydrodynamic force enables postsecretion regulation of VWF length. Mutations in VWF in von Willebrand disease contribute to and are illuminated by VWF biology. I attempt to integrate classic studies on the physiology of hemostatic plug formation into modern molecular understanding, and point out what remains to be learned.

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Crossref · 10.1182/blood-2014-05-378638

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Metadata

Authors
Springer, T. A.
Venue
Blood
Published
2014-08-28
Volume
124
Issue
9
Pages
1412-1425
ISSN
  • 0006-4971 (ISSN-L)
  • 1528-0020
OpenAlex
W1985726492
Cited by
444
References
67
FWCI
13.88
Type
journal article
Language
en
Open access
green

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Springer, T. A. (2014). von Willebrand factor, Jedi knight of the bloodstream. Blood. https://doi.org/10.1182/blood-2014-05-378638

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