VWF secretion: what's in a name?
نویسندگان
چکیده
tion in nonsplenectomized ITP patients. RCTs are particularly important in ITP because spontaneous remissions are known to occur6 and because formal evaluations of treatment efficacy that include clinically important end points—such as bleeding and quality-oflife—are needed.7 Conflict-of-interest disclosure: T.E.W. declares no competing financial interests. D.M.A. has received an operating grant from HoffmannLaRoche (Mississauga, ON), and has consulted for Amgen (Mississauga, ON). ■
منابع مشابه
THROMBOSIS AND HEMOSTASIS Intersection of mechanisms of type 2AVWD through defects in VWF multimerization, secretion, ADAMTS-13 susceptibility, and regulated storage
Type 2A VWD is characterized by the absence of large VWF multimers and decreased platelet-binding function. Historically, type 2A variants are subdivided into group 1, which have impaired assembly and secretion of VWF multimers, or group 2, which have normal secretion of VWF multimers and increased ADAMTS13 proteolysis. Type 2A VWD patients recruited through the T. S. Zimmerman Program for the ...
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Endothelial cells store the adhesive glycoprotein von Willebrand factor (VWF) in Weibel-Palade bodies (WPBs), distinctively shaped regulated secretory organelles that undergo exocytosis in response to secretagogue. A significant proportion of newly synthesized VWF is also secreted spontaneously from nonstimulated cells, through what is thought to be the constitutive secretory pathway. To learn ...
متن کاملG protein-dependent basal and evoked endothelial cell vWF secretion.
von Willebrand factor (vWF) secretion by endothelial cells (ECs) is essential for hemostasis and thrombosis; however, the molecular mechanisms are poorly understood. Interestingly, we observed increased bleeding in EC-Gα13(-/-);Gα12(-/-) mice that could be normalized by infusion of human vWF. Blood from Gα12(-/-) mice exhibited significantly reduced vWF levels but normal vWF multimers and impai...
متن کاملRegulated von Willebrand factor secretion is associated with agonist-specific patterns of cytoskeletal remodeling in cultured endothelial cells.
von Willebrand factor (vWF), an adhesive glycoprotein involved in primary hemostasis, is stored and released from endothelial secretory granules called Weibel-Palade bodies. Regulated secretion occurs in reaction either to [Ca(2+)](i)-raising agents (histamine or thrombin) or to cAMP-raising agents (epinephrine, adenosine, or forskolin). We investigated the pattern of release and the cytoskelet...
متن کاملImaging von Willebrand Factor secretion in vascular endothelial cells using live-cell imaging and correlative light and electron microscopy
Vascular endothelial cells lining the inner surface of blood vessels synthesize high molecular weight multimers of the blood coagulation protein von Willebrand factor (VWF). At the trans Golgi network, VWF multimers are folded together with the VWF propeptide (also called proregion) into tubular structures for storage in cigar-shaped organelles. These organelles, called Weibel-Palade bodies (WP...
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عنوان ژورنال:
- Blood
دوره 112 4 شماره
صفحات -
تاریخ انتشار 2008