Murine coagulation factor VIII is synthesized in endothelial cells
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چکیده
منابع مشابه
Murine coagulation factor VIII is synthesized in endothelial cells.
The primary cellular source of factor VIII (FVIII) biosynthesis is controversial, with contradictory evidence supporting an endothelial or hepatocyte origin. LMAN1 is a cargo receptor in the early secretory pathway that is responsible for the efficient secretion of factor V (FV) and FVIII to the plasma. Lman1 mutations result in combined deficiency of FV and FVIII, with levels of both factors r...
متن کاملPlenary Paper THROMBOSIS AND HEMOSTASIS Murine coagulation factor VIII is synthesized in endothelial cells
Plasma coagulation factor VIII (FVIII) is tightly associated with von Willebrand factor (VWF). Though it is well established that VWF is synthesized exclusively in endothelial cells andmegakaryocytes, the cellular source of FVIII has been debated for decades. The delayed rise of FVIII in von Willebrand disease patients infused with VWF demonstrates that VWF and FVIII do not require synthesis in...
متن کاملEndothelial cells synthesize FVIII Scientific section designation: THROMBOSIS AND HEMOSTASIS Title: Murine coagulation factor VIII is synthesized in endothelial cells
The primary cellular source of FVIII biosynthesis is controversial, with contradictory evidence supporting an endothelial or hepatocyte origin. LMAN1 is a cargo receptor in the early secretory pathway that is responsible for the efficient secretion of FV and FVIII to the plasma. Lman1 mutations result in combined deficiency of FV and FVIII, with levels of both factors reduced to ~10-15% of norm...
متن کاملFactor VIII Is Synthesized in Human Endothelial Cells, Packaged in Weibel-Palade Bodies and Secreted Bound to ULVWF Strings
The cellular synthesis site and ensuing storage location for human factor VIII (FVIII), the coagulation protein deficient in hemophilia A, has been elusive. FVIII stability and half-life is dependent on non-covalent complex formation with von Willebrand factor (VWF) to avoid proteolysis and clearance. VWF is synthesized in megakaryocytes and endothelial cells, and is stored and secreted from pl...
متن کاملSustained expansion and transgene expression of coagulation factor VIII-transduced cord blood-derived endothelial progenitor cells.
OBJECTIVE Although hemophilia A seems particularly suitable for gene therapy because even low amounts of plasma coagulation factor VIII (FVIII) provide a significant clinical benefit to the patients, the ideal target cell for recombinant FVIII expression and gene therapy approaches remains to be identified. In this study, we tested the capacity of cord blood-derived endothelial progenitor cells...
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ژورنال
عنوان ژورنال: Blood
سال: 2014
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood-2014-02-554501