Affinity chromatography of human cryosupernatants on anti-human factor VIII-Sepharose yielded a plasma devoid of detectable factor VIIIC, VIIIR:Ag, and VIIIR:WF activities. This plasma was indistinguishable from severe congenital hemophilic plasma when used as substrate in factor VIII coagulant assays.

Previously we created two strains of factor VIII-deficient mice by insertion of a neo gene into (1) the 3' end of exon 16 and (2) exon 17 of the factor VIII gene. Affected mice of both strains have no plasma factor VIII activity, yet are healthy with no spontaneous bleeding. Factor VIII-deficient females bred with affected males survive pregnancy and delivery. We used reverse transcriptase-poly...

patients with coagulation factor(s) deficiency who use coagulation therapy are susceptible to forming inhibitors against coagulation factor(s). in this survey we detected factor v and viii inhibitor in ten patients with combined deficiency of factors v and viii from north east of iran (khorassan province). it was revealed in our survey that eight patients had both factor v and factor viii inhib...

conclusions tissue engineering may open a new window in cleft palate reconstruction. stem cells and growth factors play key roles in this field. results we reported a summary of the key-points concerning cleft palate development, the genes involving this defect, current therapeutic strategies, recently novel aspects, and future advances in treatments for easy and fast understanding of the conce...

With the aim of the production of human factor VIII antigen and its corresponding antibody an epitope coding fragment of the light-chain of hFVIII, fused to a His6-tag, was isolated and over-expressed in Escherichia coli. The over-expressed hFVIII-epitope containing peptide was confirmed by its reaction with a rabbit serum directed against native hFVIII as well as antiHis6-tag antibody. An expr...

The development of anti-factor VIII antibodies is a major complication of the treatment of patients with hemophilia A. Generation of high affinity anti-factor VIII antibodies is dependent on help provided by CD4+ T cells that recognize factor VIII-derived peptides presented on class II major histocompatibility complex on the surface of antigen-presenting cells. In order to identify the immune-d...

To study the interaction between factor VIII and von Willebrand factor (vWF), binding experiments were performed using immobilized plasma vWF. Plasma was obtained from healthy donors and from patients with severe hemophilia A. For normal and hemophilic vWF, the dissociation constants (kd) for binding of factor VIII to vWF were 0.21 +/- 0.04 and 0.22 +/- 0.05 nmol/L, respectively. At saturation,...

Activated platelets release their granule content in a concentrated fashion at sites of injury. We examined whether ectopically expressed factor VIII in developing megakaryocytes would be stored in alpha-granules and whether its release from circulating platelets would effectively ameliorate bleeding in a factor VIIInull mice model. Using the proximal glycoprotein 1b alpha promoter to drive exp...

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...

Factor VIII (antihemophilic factor) has been purified approximately 500.fold from the cryoprecipitate fraction of human plasma. The isolation procedure involves adsorption of contaminants with Al(OH),, column chromatography on tricalcium citrate-cellulose, precipitation with concanavalin A, and an agarose gel filtration step. The linal product is homogeneous when examined by zone electrophoresi...