Ovine primary fetal fibroblasts were cotransfected with a neomycin resistance marker gene (neo) and a human coagulation factor IX genomic construct designed for expression of the encoded protein in sheep milk. Two cloned transfectants and a population of neomycin (G418)-resistant cells were used as donors for nuclear transfer to enucleated oocytes. Six transgenic lambs were liveborn: Three prod...

Thrombosis and transmission of viral diseases are the principal adverse effects of current replacement therapy for factor IX deficiency when using heat-treated concentrates of vitamin K-dependent coagulation factors. More highly purified factor IX preparations could decrease the risk of disease transmission, reduce patient exposure to allogeneic proteins, and reduce the risk of thrombosis. In t...

Previous studies have shown that factor IX and its activated form, factor IXa, bind to cultured vascular endothelial cells and that cell-bound factor IXa retains its procoagulant activity. The present studies provide evidence that factor IX bound to cultured bovine aortic endothelial cells can be activated. Factor IX activation was assessed by finding cleavage of the factor IX molecule on NaDod...

The effect of two arginine-specific cysteine proteinases (gingipains R) from Porphyromonas gingivalis, an aetiological factor of adult periodontitis, on the activation of human factor IX was investigated in the presence of ethylene glycol, an activity enhancer of activated factor IX (factor IXa), with the use of a fluorogenic oligopeptide substrate. Each gingipain R rapidly activated factor IX ...

Germline mutations cause or predispose to most disease. Hemophilia B is a useful model for studying the underlying pattern of recent germline mutations in humans because the observed pattern of mutation in factor IX more closely reflects the underlying pattern of mutation than the observed pattern for many other genes. In addition, it is possible to identify and correct for biases inherent in a...

abstract  introduction: during extrinsic coagulation pathway, a complex is developed between factor vii, calcium and tissue factor (a cell membrane lipoprotein that is exposed after cell injury). factor vii needs calcium and vitamin k for its biologic activation. coronary artery disease (cad) can be induced by increased level and activity of the coagulation factors vii, viii and ix. in postmeno...

A persistent low-titer factor IX Inhibitor was discovered in a patIent with severe hemophllia B. The Inhibitor was very likely an immunoglobulin, since it was present in serum, was not dialyzable, retained its potency after heating to 56#{176}C, and was bound by staphylococcal protein A (SPA). When the hemophilia B patient with the inhibItor was given therapeutic infusions of factor Ix concentr...