Intracellular trafficking of factor VIII to von Willebrand factor storage granules.

Intracellular trafficking of factor VIII to von Willebrand factor storage granules.

In plasma, von Willebrand factor (vWf) associates with Factor VIII (FVIII); however, the site at which these proteins first interact has not been defined. Administration of 1-desamino-8-D-arginine vasopressin (DDAVP) causes a rapid, concomitant elevation in plasma levels of both vWf and FVIII, suggesting the existence of a DDAVP-releasable storage pool for both proteins. To determine whether vW...

Intracellular cotrafficking of factor VIII and von Willebrand factor type 2N variants to storage organelles.

Weibel-Palade bodies (WPBs) are the endothelial storage organelles that are formed upon von Willebrand factor (VWF) expression. Apart from VWF, WPBs contain a variety of hemostatic and inflammatory proteins. Some of these are thought to be targeted to WPBs by directly interacting with VWF in the secretory pathway. Previous studies have demonstrated that coexpression of factor VIII (FVIII) with ...

Intracellular co-trafficking of factor VIII and von Willebrand factor type 2N variants to storage organelles Running head: FVIII and VWF type 2N co-trafficking

THROMBOSIS AND HEMOSTASIS Intracellular cotrafficking of factor VIII and von Willebrand factor type 2N variants to storage organelles

Weibel-Palade bodies (WPBs) are the endothelial storage organelles that are formed upon von Willebrand factor (VWF) expression. Apart from VWF, WPBs contain a variety of hemostatic and inflammatory proteins. Some of these are thought to be targeted to WPBs by directly interacting with VWF in the secretory pathway. Previous studies have demonstrated that coexpression of factor VIII (FVIII) with ...

Factor VIII/von Willebrand factor protein. Galactose a cryptic determinant of von Willebrand factor activity.

The normal Factor VIII/von Willebrand factor protein has the ability to agglutinate or aggregate normal platelets in the presence of ristocetin (von Willebrand factor activity). Removal of greater than 95% of the sialic acid from this protein by neuraminidase did not affect the von Willebrand factor or procoagulant activity. However, oxidation of the penultimate galactose of the asialo Factor V...