von Willebrand factor proteolytic processing and multimerization precede the formation of Weibel-Palade bodies

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von Willebrand factor proteolytic processing and multimerization precede the formation of Weibel-Palade bodies.

We investigated the intracellular site of pro-von Willebrand factor (pro-vWF) cleavage and multimerization, as well as the fate of the propolypeptide (von Willebrand antigen II) after cleavage. Analysis of subcellular fractions of endothelial cells metabolically labeled with sulfate showed that both cleavage and covalent multimerization occur after sulfation and precede the formation of Weibel-...

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Von Willebrand disease and Weibel-Palade bodies.

Von Willebrand factor (VWF) is a pivotal haemostatic protein mediating platelet adhesion to injured endothelium and carrying coagulation factor VIII (FVIII) in the circulation to protect it from premature clearance. Apart from the roles in haemostasis, VWF drives the formation of the endothelial cell specific Weibel-Palade bodies (WPBs), which serve as a regulated storage of VWF and other throm...

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Actomyosin II contractility expels von Willebrand factor from Weibel–Palade bodies during exocytosis

The study of actin in regulated exocytosis has a long history with many different results in numerous systems. A major limitation on identifying precise mechanisms has been the paucity of experimental systems in which actin function has been directly assessed alongside granule content release at distinct steps of exocytosis of a single secretory organelle with sufficient spatiotemporal resoluti...

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Immunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cells

Immunofluorescence staining of cultured human umbilical vein endothelial cells has shown the presence of von Willebrand protein in the perinuclear region, in small rodlike structures through the cytoplasm, and on filaments of the extracellular matrix. Nonendothelial cells showed no staining with anti-von Willebrand protein antiserum. At the light microscope level, immunoperoxidase treatment of ...

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Von-Willebrand-Erkrankung und Weibel-Palade-Körperchen

Von Willebrand factor (VWF) is a pivotal haemostatic protein mediating platelet adhesion to injured endothelium and carrying coagulation factor VIII (FVIII) in the circulation to protect it from premature clearance. Apart from the roles in haemostasis, VWF drives the formation of the endothelial cell specific Weibel-Palade bodies (WPBs), which serve as a regulated storage of VWF and other throm...

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ژورنال

عنوان ژورنال: Blood

سال: 1994

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood.v83.12.3536.3536