The Von Willebrand Factor Propeptide (VWFpp) Traffics an Unrelated Protein to Storage

The von Willebrand factor propeptide (VWFpp) traffics an unrelated protein to storage.

The von Willebrand factor (VWF) propeptide (VWFpp) is critical for the targeting of VWF multimers to storage granules. VWFpp alone efficiently navigates the storage pathway in AtT-20 and endothelial cells and chaperones mature VWF multimers to storage granules when the two proteins are expressed in cis or in trans. To further define the role of VWFpp in granular sorting, we examined its ability...

Von Willebrand Factor propeptide (VWFpp) binding to VWF D’D3 domain attenuates platelet activation and adhesion

Von Willebrand Factor Propeptide Enhances Il-8-induced Neutrophil Adhesion

von Willebrand factor propeptide: biology and clinical utility.

von Willebrand factor (VWF) is a large multimeric glycoprotein that mediates the attachment of platelets to damaged endothelium and also serves as the carrier protein for coagulation factor VIII (FVIII), protecting it from proteolytic degradation. Quantitative or qualitative defects in VWF result in von Willebrand disease (VWD), a common inherited bleeding disorder. VWF is synthesized with a ve...

Critical independent regions in the VWF propeptide and mature VWF that enable normal VWF storage.

Von Willebrand factor (VWF) is synthesized in endothelial cells, where it is stored in Weibel-Palade bodies. Administration of 1-desamino-8-D-arginine-vasopressin (DDAVP) to patients with type 1 von Willebrand disease and to healthy individuals causes a rapid increase in plasma VWF levels. This increase is the result of stimulated release of VWF from Weibel-Palade bodies in certain beds of endo...