... factor VIII (FVIII)-binding region. Yet, von Willebrand disease (VWD) Type 2 Normandy (2N) mutations, associated with impaired FVIII interaction, have been identified in C8_3-TIL3-E3. We now assessed the role of the VWF (sub)domains for FVIII binding using isolated D′, D3 and monomeric C-terminal...

... mutations are the most common mutations in inherited diseases; however, missense mutations that generate extra, non-native N-glycosylation sites have not been well characterized. Coagulation factor VIII (FVIII) contains five consensus N-glycosylation sites outside its functionally dispensable B domain. We...

...Dionysios Pantazatos; Christopher R. Gessner; Virgil L. Woods, Jr; Gary E. Gilbert Factor VIII enhances the catalytic activity of Factor IXa in a membrane-bound enzyme complex and both proteins are necessary to prevent haemophilia. Tandem lectin-like C domains mediate the membrane binding of Factor...

... and Arg 1721 . 1 To whom correspondence should be addressed (email [email protected] ). 3 1 2013 19 3 2013 25 3 2013 25 3 2013 © The Authors Journal compilation © 2013 Biochemical Society 2013 F (Factor) VIII is a plasma protein that is decreased...

...Valerie A. Novakovic; David B. Cullinan; Hironao Wakabayashi; Philip J. Fay; James D. Baleja; Gary E. Gilbert Factor VIII functions as a cofactor for Factor IXa in a membrane-bound enzyme complex. Membrane binding accelerates the activity of the Factor VIIIa–Factor IXa complex approx. 100000-fold...

... of Factor VIIIa was examined by employing recombinant Factor VIIIa molecules where residues 336 and 562 were replaced with alanine and/or glutamine. Stably expressed Factor VIII proteins were activated by thrombin and resultant Factor VIIIa was reacted at high concentration with APC to minimize cofactor...

... blood coagulation Factor VIII Factor IX Factor X mathematical modelling platelet fIXa is an M r 44000 two-chain vitamin K-dependent serine protease capable of hydrolysing the Arg 194 -Ile 195 peptide bond in the fX molecule, which leads to its activation [ 5 ]. Although this reaction can...

...R. De CRISTOFARO; V. De FILIPPIS Activation of factor VIII (FVIII) by thrombin plays a fundamental role in the amplification of the coagulation cascade and takes place through specific proteolytic cleavages at Arg 372 , Arg 740 and Arg 1689 . Full FVIII activation requires cleavage at Arg 372...

... of thrombin, including fibrinogen, factor VIII, factor XIII and protein C. In addition, these proteins were bound to anhydrothrombin–agarose in a reversible manner. The K d values for factor VIII, fibrinogen, factor XIII and protein C were 1.2×10 -8 , 4.4×10 -8 , 2.8×10 -7 and 8.1×10 -5 M, respectively. Thus...

.... The requirement for Lys at position 316 for FX activation was also evident in the presence of the cofactor activated Factor VIII, although to a lesser extent than in its absence. These data demonstrate that Lys 316 specifically determines the reactivity of FIXa towards its natural substrate FX...