نتایج جستجو برای: fviii

تعداد نتایج: 1331  

2013
R T PETERS G TOBY Q LU T LIU J D KULMAN S C LOW A J BITONTI G F PIERCE

BACKGROUND Hemophilia A results from a deficiency in factor VIII activity. Current treatment regimens require frequent dosing, owing to the short half-life of FVIII. A recombinant FVIII-Fc fusion protein (rFVIIIFc) was molecularly engineered to increase the half-life of FVIII, by 1.5-2-fold, in several preclinical animal models and humans. OBJECTIVE To perform a biochemical and functional in ...

Journal: :Blood 1995
D Scandella G E Gilbert M Shima H Nakai C Eagleson M Felch R Prescott K J Rajalakshmi L W Hoyer E Saenko

The finding that human factor VIII (fVIII) inhibitor antibodies with C2 domain epitopes interfere with the binding of fVIII to phosphatidylserine (PS) suggested that this is the mechanism by which they inactivate fVIII. We constructed a recombinant C2 domain polypeptide and demonstrated that it bound to all six human inhibitors with fVIII light chain specificity. Thus, some antibodies within th...

2017
Jerry Powell

Thromb Haemost 2008; 100: 365–366 Haemophilia A is an X-linked bleeding disorder caused by a deficiency of factor VIII (FVIII), a necessary cofactor for the generation of thrombin. The life expectancy of a child born with severe haemophlia A has improved dramatically over the past 30 years, from less than 20 years to nearly normal, primarily due to the development of FVIII concentrates, but als...

Journal: :Blood 1998
K Amano R Sarkar S Pemberton G Kemball-Cook H H Kazazian R J Kaufman

Factor VIII (FVIII) is the protein defective in the bleeding disorder hemophilia A. Approximately 5% of hemophilia A patients have normal amounts of a dysfunctional FVIII protein and are termed cross-reacting material (CRM)-positive. The majority of genetic alterations that result in CRM-positive hemophilia A are missense mutations within the A2-domain. To determine the mechanistic basis of the...

Journal: :Blood 1982
J D Levine J M Harlan L A Harker M L Joseph R B Counts

We have examined the effects of purified human alpha-thrombin on factor VIII antigen (FVIII-Ag) release by human umbilical vein endothelial cells in culture. Alpha-thrombin induced a time and dose-dependent release of FVIII-Ag into supernatant medium. Alpha-thrombin-mediated FVIII-Ag release was not dependent on protein synthesis and was observed in both serum-free and serum-containing media. F...

2015
Daniela Dalm Jesus G. Galaz-Montoya Jaimy L. Miller Kirill Grushin Alex Villalobos Alexey Y. Koyfman Michael F. Schmid Svetla Stoilova-McPhie

Membrane-bound Factor VIII (FVIII) has a critical function in blood coagulation as the pro-cofactor to the serine-protease Factor IXa (FIXa) in the FVIIIa-FIXa complex assembled on the activated platelet membrane. Defects or deficiency of FVIII cause Hemophilia A, a mild to severe bleeding disorder. Despite existing crystal structures for FVIII, its membrane-bound organization has not been reso...

2004
Christina Hausl Rafi U. Ahmad Hans Peter Schwarz Eva M. Muchitsch Peter L. Turecek Friedrich Dorner Birgit M. Reipert

Memory B cells are responsible for the rapidly emerging antibody response after antigen reexposure. The signals required for the restimulation of memory B cells have not been fully explained. We used a murine model of anti–factor VIII (FVIII) antibody responses in hemophilia A to study the requirements for the restimulation of FVIII-specific memory B cells and their differentiation into anti-FV...

Journal: :Blood 2004
Brian D Brown Chang Xin Shi Sandra Powell David Hurlbut Frank L Graham David Lillicrap

Two helper-dependent (HD) adenoviral vectors encoding a canine factor VIII B-domain-deleted transgene (cFVIII) were constructed and evaluated in 4 hemophilia A dogs. One vector was regulated by the cytomegalovirus (CMV) promoter (HD-CMV-cFVIII), while the other vector contained a tissue-restricted promoter comprised of the human FVIII proximal promoter with an upstream concatemer of 5 hepatocyt...

Journal: :Blood 2009
Baowei Peng Peiqing Ye David J Rawlings Hans D Ochs Carol H Miao

One major obstacle in gene therapy is the generation of immune responses directed against transgene product. Five consecutive anti-CD3 treatments concomitant with factor VIII (FVIII) plasmid injection prevented the formation of inhibitory antibodies against FVIII and achieved persistent, therapeutic levels of FVIII gene expression in treated hemophilia A mice. Repeated plasmid gene transfer is ...

Journal: :Blood 2004
Jean Guy G Gilles Sabrina C Grailly Marc De Maeyer Marc G Jacquemin Luc P VanderElst Jean-Marie R Saint-Remy

Factor VIII (FVIII) administration elicits specific inhibitory antibodies (Abs) in about 25% of patients with hemophilia A. The majority of such Abs reacts with FVIII C2 domain. mAbBO2C11 is a high-affinity human monoclonal antibody (mAb) directed toward the C2 domain, which is representative of a major class of human FVIII inhibitors. Anti-idiotypic Abs were raised to mAbBO2C11 to establish th...

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