OBJECTIVE Poor prognosis of sepsis is associated with bacterial lipopolysaccharide (LPS)-induced intravascular inflammation, microvascular thrombosis, thrombocytopenia, and disseminated intravascular coagulation. Platelets are critical for thrombosis, and there has been increasing evidence of the importance of platelets in endotoxemia. The platelet adhesion receptor, the glycoprotein Ib-IX comp...

Introduction Haemophilia B is an X-linked disorder resulting in coagulation factor IX (FIX) deficiency that is treated with the administration of exogenous FIX obtained from plasma of healthy donors or by a DNA recombinant technique1. The development of an inhibitor antibody against exogenous FIX is a serious complication and occurs in 1-3% of patients with haemophilia B and in 25-30% of patien...

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...

Inhibitors of clotting factors occuring in humans are often antibody molecules synthesized in response to exogeneous proteins used in replacement therapy. Extensive studies of inhibitors to factor VIII indicate such antibodies may be monoclonal or polyclonal in nature. To date, only one factor IX inhibitor has been subjected to detailed immunochemical analysis and it appears to be a monoclonal ...

Recent investigations have suggested that the activation of factor IX by factor VII/tissue factor may be an important alternative route to the generation of factor Xa. Accordingly, we have compared the tissue factor-dependent activation of tritium-labeled factor IX and factor X in a human plasma system and have studied the role of proteases known to stimulate factor VII activity. Plasma was def...

To probe the molecular interactions of factor Xl we have prepared two monoclonal antibodies (MoAbs; 5F7 and 3C1 ), each of which binds the heavy chain of reduced and alkylated factor Xla. Competitive solid phase radioimmunoassay (RIA) binding studies revealed that 5F7 and 3C1 are directed against different epitopes within factor Xl. One antibody (5F7) blocked the surface-mediated proteolytic ac...

hemophilia b is factor ix deficiency and is inherited as x-linked recessive disorder. the subject of carrier detection in hemophilias has received new impetus in the last several years. analysis of factor ix gene polymorphisms is considered the best approach for prenatal diagnosis and hemophilia b carrier detection, if the identification of the gene mutation is possible. allele frequencies of t...

A procedure is presented for the purification of Factor IX from human plasma. The final product is homogeneous as judged by disc gel electrophoresis and sodium dodecyl sulfate gel electrophoresis. Furthermore, it is completely free of other coagulation component activities. Factor IX is converted to its enzymatically active form by the addition of small quantities of Factor IXa in the presence ...

The study of coagulation factors has been rapidly advanced by studies performed in genetically engineered mouse strains. Investigation of factor IX (FIX) has benefited from excellent gene-deleted mouse models that recapitulate many of the features of human haemophilia B. Moreover, advanced positional cloning techniques and availability of technology to allow not only knock-out mice, but also kn...