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- W2011334787 endingPage "5043" @default.
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- W2011334787 abstract "Weibel-Palade bodies (WPBs) are elongated secretory organelles specific to endothelial cells that contain von Willebrand factor (VWF) and a variety of other proteins that contribute to inflammation, angiogenesis, and tissue repair. The remarkable architecture of WPBs is because of the unique properties of their major constituent VWF. VWF is stored inside WPBs as tubules, but on its release, forms strikingly long strings that arrest bleeding by recruiting blood platelets to sites of vascular injury. In recent years considerable progress has been made regarding the molecular events that underlie the packaging of VWF multimers into tubules and the processes leading to the formation of elongated WPBs. Mechanisms directing the conversion of tightly packaged VWF tubules into VWF strings on the surface of endothelial cells are starting to be unraveled. Several modes of exocytosis have now been described for WPBs, emphasizing the plasticity of these organelles. WPB exocytosis plays a role in the pathophysiology and treatment of von Willebrand disease and may have impact on common hematologic and cardiovascular disorders. This review summarizes the major advances made on the biogenesis and exocytosis of WPBs and places these recent discoveries in the context of von Willebrand disease." @default.
- W2011334787 created "2016-06-24" @default.
- W2011334787 creator A5017437441 @default.
- W2011334787 creator A5017838878 @default.
- W2011334787 creator A5044510198 @default.
- W2011334787 creator A5071555653 @default.
- W2011334787 creator A5072522782 @default.
- W2011334787 date "2011-05-12" @default.
- W2011334787 modified "2023-10-10" @default.
- W2011334787 title "Functional architecture of Weibel-Palade bodies" @default.
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