background: inhibitory antibody to exogenous factor viii (fviii) is a major complication of hemophilia treatment. this study was conducted to determine the prevalence of inhibitor antibody directed against fviii.methods: from may 2010 to may 2011, 52 patients with severe hemophilia a admitted in amirkola children’s hospital were evaluated. those who had abnormal mixing study, antibody against f...

Hemophilia A is caused by a deficiency in coagulation factor VIII (FVIII) and predisposes to spontaneous bleeding that can be life-threatening or lead to chronic disabilities. It is well suited for gene therapy because a moderate increase in plasma FVIII concentration has therapeutic effects. Improved retroviral vectors expressing high levels of human FVIII were pseudotyped with the vesicular s...

Abstract Hemophilia A is an X-linked bleeding disorder resulting in events due to insufficient levels of factor VIII (FVIII). The most significant complication the management hemophilia formation polyclonal neutralizing IgG (i.e., inhibitors) which impede FVIII procoagulant activity. In a (FVIII KO) murine model injected with multiple doses FVIII, FVIII-specific IgM were found persist despite e...

Essentials Factor VIII inhibitors are the most serious complication in patients with hemophilia A. Aggregates in biopharmaceutical products are an immunogenic risk factor. Aggregates were identified in recombinant full-length factor VIII products. Aggregates in recombinant factor VIII products are identified by analytical ultracentrifugation. SUMMARY Background The development of inhibitory a...

Abstract Deficiency of the procoagulant protein factor VIII (FVIII) is defining characteristic for individuals with inherited X-linked bleeding disorder hemophilia A. These require intravenous infusions FVIII to treat events, however ~30% develop neutralizing alloantibodies (i.e., inhibitors) against exogenous FVIII. Transcriptome analysis FVIII-stimulated peripheral blood mononuclear cells in ...

Factor VIII belongs to the coagulation cascade and is expressed as a long pre-protein (mature form, 2351 amino acids long). FVIII deficient or defective in hemophilic A patients, who need be treated with hemoderivatives recombinant substitutes, i.e., biologic drugs. The interaction between von Willebrand factor (VWF) influences pharmacokinetics of medications. In vivo, full-length (FL-FVIII) se...

Background and Aims: Hemophilia is a rare autoimmune disorder caused by autoantibodies directed in the majority of the cases against clotting factor VIII (FVIII). FVIII is extracted from human plasma or engineered from mammalian cell cultures using recombinant DNA technology. In Iran, most of the used FVIII is prepared from human plasma in Iranian Blood Transfusion Organization. It was seen imp...

Processing by the proprotein convertase furin is believed to be critical for the biological activity of multiple proteins involved in hemostasis, including coagulation factor VIII (FVIII). This belief prompted the retention of the furin recognition motif (amino acids 1645-1648) in the design of B-domain-deleted FVIII (FVIII-BDD) products in current clinical use and in the drug development pipel...

Anti-Factor VIII (FVIII) antibodies were prepared by a combination of salt precipitation, gel filtration chromatography, and specific adsorption over insolubilized FVIII from the serum of 10 healthy subjects with normal levels of FVIII. Antibody specificity was confirmed by the capacity to recognize soluble and insolubilized FVIII and to neutralize FVIII cofactor activity in FX activation. Epit...

Factor VIII (FVIII) functions as a cofactor for factor IXa in the contact coagulation pathway and circulates in a protective complex with von Willebrand factor (VWF). Plasma FVIII activity is strongly influenced by environmental and genetic factors through VWF-dependent and -independent mechanisms. Single nucleotide polymorphisms (SNPs) of the coding and promoter sequence in the FVIII gene have...