156. Cryopreservation of recombinant antihemophilic Factor VIII. Effect of biopolymers. Nelly M. Tsvetkova, Omkar Joshi, Arnaud Desponds, D.Q. Wang, Paul Wu, Bayer HealthCare LLC, Berkeley, CA, USA Recombinant Factor VIII, an essential coagulation factor in the blood, is one of the most complex proteins manufactured today. Currently the only therapy for hemophilia A is life-long administration ...

The development of inhibitory 'allo' antibodies to a deficient coagulation factor is arguably now the most severe and important complication of clotting factor concentrate exposure in haemophilia and other congenital coagulation disorders. Furthermore, development of an inhibitor to the factor VIII or factor IX transgene product remains a significant concern in gene therapy protocols for haemop...

Comparative studies of coagulation factors in arterial and venous blood were made in 14 normal subjects and in 4 patients with arterial or ventricular septal defect. No difference was found at the level of prothrombin, factor V, factor VII/X, retraction, factor XIII, and platelet count. Fibrinogen and factor VIII were significantly low in the venous blood. The platelet count was higher in the c...

Cryo-electron microscopy (Cryo-EM)(1) is a powerful approach to investigate the functional structure of proteins and complexes in a hydrated state and membrane environment(2). Coagulation Factor VIII (FVIII)(3) is a multi-domain blood plasma glycoprotein. Defect or deficiency of FVIII is the cause for Hemophilia type A - a severe bleeding disorder. Upon proteolytic activation, FVIII binds to th...

BACKGROUND Hemophilia A is caused by heterogeneous mutations in F8. Coagulation factor VIII (FVIII), the product of F8, is composed of multiple domains designated A1-A2-B-A3-C1-C2. FVIII is known to interact with diverse proteins, and this characteristic may be important for hemostasis. However, little is known about domain-specific functions or their specific binding partners. METHODS To det...

Animal models of the bleeding disorder, hemophilia A, have been an integral component of the biopharmaceutical development process and have facilitated the development of recombinant coagulation factor VIII (fVIII) products capable of restoring median survival of persons with hemophilia A to that of the general population. However, there remain several limitations to recombinant fVIII as a biot...

The human coagulation factor VIII (FVIII) is essential in the intrinsic pathway of blood coagulation and circulates mainly as a non-covalently bound complex with the von Willebrand factor (VWF). This complex (FVIII/VWF) protects FVIII from degradation and cellular uptake, although no biological role has been identified yet for this complex. The FVIII/VWF complex was purified from a healthy dono...