Red Blood Cells are Appropriate Carrier for Coagulation Factor VIII
نویسندگان
چکیده
منابع مشابه
Helical organization of blood coagulation factor VIII on lipid nanotubes.
Cryo-electron microscopy (Cryo-EM)(1) is a powerful approach to investigate the functional structure of proteins and complexes in a hydrated state and membrane environment(2). Coagulation Factor VIII (FVIII)(3) is a multi-domain blood plasma glycoprotein. Defect or deficiency of FVIII is the cause for Hemophilia type A - a severe bleeding disorder. Upon proteolytic activation, FVIII binds to th...
متن کاملMurine 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 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...
متن کاملMapping the binding region on the low density lipoprotein receptor for blood coagulation factor VIII.
Low density lipoprotein receptor (LDLR) was shown to mediate clearance of blood coagulation factor VIII (FVIII) from the circulation. To elucidate the mechanism of interaction of LDLR and FVIII, our objective was to identify the region of the receptor necessary for binding FVIII. Using surface plasmon resonance, we found that LDLR exodomain and its cluster of complement-type repeats (CRs) bind ...
متن کاملBioengineering of coagulation factor VIII for improved secretion.
Factor VIII (FVIII) functions as a cofactor within the intrinsic pathway of blood coagulation. Quantitative or qualitative deficiencies of FVIII result in the inherited bleeding disorder hemophilia A. Expression of FVIII (domain structure A1-A2-B-A3-C1-C2) in heterologous mammalian systems is 2 to 3 orders of magnitude less efficient compared with other proteins of similar size compromising rec...
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ژورنال
عنوان ژورنال: Cardiovascular & Hematological Disorders-Drug Targets
سال: 2020
ISSN: 1871-529X
DOI: 10.2174/1871529x19666190918141859