Background: Hemophilia B is an X-linked hereditary disorder of blood coagulation system which is caused by factor IX (FIX) deficiency. Factor IX is a plasma glycoprotein that participates in the coagulation process leading to the generation of fibrin. Replacement of factor IX with plasma-derived or recombinant factor IX is the conventional treatment for hemophilia B to raise the factor IX le...

background: hemophilia b is an x-linked hereditary disorder of blood coagulation system which is caused by factor ix (fix) deficiency. factor ix is a plasma glycoprotein that participates in the coagulation process leading to the generation of fibrin. replacement of factor ix with plasma-derived or recombinant factor ix is the conventional treatment for hemophilia b to raise the factor ix level...

The majority of cases of human hemophilia B are the result of missense mutations in the coagulation factor IX gene and defective circulating factor IX is detectable in most patients. The available mouse factor IX knockout models of hemophilia B (FIXKO mouse) reproduce the bleeding phenotype of human hemophilia B, but because the models produce no factor IX they fail to reproduce the dominant hu...

The majority of cases of human hemophilia B are the result of missense mutations in the coagulation factor IX gene and defective circulating factor IX is detectable in most patients. The available mouse factor IX knockout models of hemophilia B (FIXKO mouse) reproduce the bleeding phenotype of human hemophilia B, but because the models produce no factor IX they fail to reproduce the dominant hu...

background: eukaryotic proteins generally have signal peptides, which are not only crucial for their secretion efficiencies but are important for their expression levels. the coagulation factor ix (fix) is a glycoprotein that plays a fundamental role in a blood coagulation pathway. reduced levels or dysfunctional fix are associated with hemophilia b. to improve the hfix secretion efficiency in ...

many investigations have proved relations between abo blood groups with some diseases and factor viii and von willebrand level in plasma. in this study we investigated a relation between abo blood groups and factor viii and ix inhibitors in 102 patients with haemophilia a and 48 patients with haemophilia b. the assay of inhibitor was done by bethesda method. there were no relation between abo b...

INTRODUCTION Gene therapy could prevent bleeding in hemophilia. However, antibodies could inhibit coagulation, while cytotoxic T lymphocytes could destroy modified cells. The immaturity of the newborn immune system might prevent these immune responses from occurring after neonatal gene therapy. MATERIALS AND METHODS Newborn dogs, cats, or mice were injected intravenously with a retroviral vec...

We have determined the 2905 nucleotide sequence of the rhesus macaque factor IX complementary DNA (cDNA) and found it to be greater than 95% identical to that of the human factor IX cDNA. The cDNA has a large 3' untranslated region like the human cDNA, but unlike the human cDNA has two polyadenylation sites 224 nucleotides apart that are used for transcription of the messenger RNA. The deduced ...

Mouse primary skin fibroblasts were infected with a recombinant retrovirus containing human factor IX cDNA. Bulk infected cells capable of synthesizing and secreting biologically active human factor IX protein were embedded in collagen, and the implant was grafted under the epidermis. Sera from the transplanted mice contain human factor IX protein for at least 10-12 days. Loss of immunoreactive...

in this study, anion-exchange chromatography was used to purify factor vii and factor ix from prothrombin complex (ppsb), which contains coagulation factors ii, vii, ix and x. for this purpose, deae-sepharose cl-6b gel , pharmacia column xk-26 , high flow rate and two stepwise gradients with phosphate citrate buffer were used. the yield of the two lyophylized products, factor vii and factor ix ...