Bioengineering coagulation factors for improved hemophilia treatments
نویسندگان
چکیده
منابع مشابه
Bioengineering coagulation factors for improved hemophilia treatments☆
The implementation of prophylaxis with regular factor VIII (FVIII) replacement for patients with hemophilia radically changed the clinical presentation of this condition, from a disease characterized by progressive disabling musculoskeletal complications, to one compatible with an active and virtually normal lifestyle. In Brazil, although most adults with severe hemophilia still suffer the impa...
متن کاملBioengineering of coagulation factor VIII for improved secretion.
Factor VIII (FVIII) functions as a cofactor within the intrinsic pathway of blood coagulation. Quantitative or qualitative deficiencies of FVIII result in the inherited bleeding disorder hemophilia A. Expression of FVIII (domain structure A1-A2-B-A3-C1-C2) in heterologous mammalian systems is 2 to 3 orders of magnitude less efficient compared with other proteins of similar size compromising rec...
متن کاملHEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Bioengineering of coagulation factor VIII for improved secretion
Factor VIII (FVIII) functions as a cofactor within the intrinsic pathway of blood coagulation. Quantitative or qualitative deficiencies of FVIII result in the inherited bleeding disorder hemophilia A. Expression of FVIII (domain structure A1-A2-BA3-C1-C2) in heterologous mammalian systems is 2 to 3 orders of magnitude less efficient compared with other proteins of similar size compromising reco...
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Factor IX is a zymogen enzyme of the blood coagulation cascade. Inherited absence or deficit of the IX functional factor causes bleeding disorder hemophilia B, which requires constant protein replacement therapy. Reviewed herein are the current state in the manufacturing of FIX, improved variants of the recombinant protein for therapy, transgenic organisms for obtaining FIX, and the advances in...
متن کاملBlood coagulation in hemophilia A and hemophilia C.
Tissue factor (TF)-induced coagulation was compared in contact pathway suppressed human blood from normal, factor VIII-deficient, and factor XI-deficient donors. The progress of the reaction was analyzed in quenched samples by immunoassay and immunoblotting for fibrinopeptide A (FPA), thrombin-antithrombin (TAT), factor V activation, and osteonectin. In hemophilia A blood (factor VIII:C <1%) tr...
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ژورنال
عنوان ژورنال: Revista Brasileira de Hematologia e Hemoterapia
سال: 2016
ISSN: 1516-8484
DOI: 10.1016/j.bjhh.2016.05.001