Coagulation proteins factor

Factor VIII Coagulation proteins hemophilia A treatment 300 3 X 10- ... 

Zymogen is a precursor protein that is converted to an active protease when one or more of its peptide bonds are cleaved. Zymogens involved in coagulation include factors II (prothrombin), VII, IX, X, and XI. 

Vitamin K is an essential factor in the production of coagulation proteins within the liver. Elevated clotting times from decreased protein synthesis are indistinguishable from those produced by low vitamin K levels caused by malnutrition or poor intestinal absorption. Vitamin K (phytonadione) 10 mg subcutaneously daily for 3 days can help to establish whether the prolonged bleeding time results from loss of synthetic function in the liver or vitamin K deficiency. 

Hemophilia A and B are coagulation disorders that result from defects in the genes encoding for plasma coagulation proteins. Hemophilia A (classic hemophilia) is caused by the deficiency of factor VIII, and hemophilia B (Christmas disease) is caused by the deficiency of factor IX. The incidences of hemophilia A and B are estimated at 1 in 5000 and 1 in 30,000 male births, respectively. Both types of hemophilia are evenly distributed across all ethnic and racial groups.1... 

Intrinsic pathway All the protein factors necessary for coagulation are present in circulating blood. Clot formation may take several minutes and is initiated by activation of factor XII. 

Antibodies directed against (32 -GPI further enrich the protein on the cell surface but these promote a p38 Map-kinase signalling cascade which results in increased expression of tissue factor (TF) and reduced expression of thrombomodulin on the surface of cells. TF is a major initiator of coagulation and increased levels of TF expression have been measured on endothelial cells treated with anti-phospholipid antibodies and on monocytes both ex vivo and in vivo (Yasuda et al., 2005 Lopez-lira et al., 2005, Lopez-Pedrera et al., 2006). Thrombomodulin is a potent anti-coagulant protein which limits activation of thrombin, so the net result of circulating anti-phospholipid antibodies is to usurp the anti-coagulative, protective mechanism and initiate a pro-coagulation cascade. 

The first therapeutic recombinant DNA-derived coagulation protein licensed by the FDA was factor VIII for treatment of hemophilia A in 1992 [38]. This step forward was a landmark in hemophilia therapy. This biotechnology process reduced the theoretical risk of human-derived viruses and seemed to provide for an unlimited market supply although other human and animal proteins were often used in the manufacturing and formulation of many recombinant... 

Coagulation protein concentrates became available in the 1970s, a significant step in the prevention and management of bleeding. The factors that are currently available are factor VII, factor Vila, factor VIII, factor IX, factor XI, and factor XIII. There is also a factor VIII inhibitor bypassing factor, activated prothrombin... 

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