Therapeutic factor VIII is highly immunogenic. Despite intensive research in the last decades, the reasons why 5-30% of patients with hemophilia A (of all severities) develop inhibitory anti-factor VIII antibodies (inhibitors) following replacement ...
Aditi Varthaman+1 more
doaj +1 more source
Pre-clinical evaluation of an enhanced-function factor VIII variant for durable hemophilia A gene therapy in male mice. [PDF]
Durable factor VIII expression that normalizes hemostasis is an unrealized goal of hemophilia A adeno-associated virus-mediated gene therapy. Trials with initially normal factor VIII activity observed unexplained year-over-year declines in expression ...
Sternberg AR+4 more
europepmc +2 more sources
Safety and efficacy of BAY 94‐9027, a prolonged‐half‐life factor VIII
Essentials Recombinant factor VIII BAY 94‐9027 conjugates in a site‐specific manner with polyethylene glycol. BAY 94‐9027 was given to patients with severe hemophilia A as prophylaxis and to treat bleeds. BAY 94‐9027 prevented bleeds at dose intervals up
M. Reding+10 more
semanticscholar +1 more source
Acquired factor VIII deficiency after consuming the dried gallbladder of a cobra, Naja naja [PDF]
Acquired factor VIII deficiency is very rare, often fatal. It is associated with pregnancy, autoimmune diseases, malignancy, and drugs, although no underlying cause is found in 50%. A 49-year-old male was referred with right shoulder bruising.
Brown+14 more
core +2 more sources
Factor VIII C-domains are believed to have specific functions in cofactor activity and in interactions with von Willebrand factor. We have previously shown that factor VIII is co-targeted with von Willebrand factor to the Weibel-Palade bodies in blood ...
Eduard H.T.M. Ebberink+7 more
doaj +1 more source
The 1.7 Å X-ray crystal structure of the porcine factor VIII C2 domain and binding analysis to anti-human C2 domain antibodies and phospholipid surfaces. [PDF]
The factor VIII C2 domain is essential for binding to activated platelet surfaces as well as the cofactor activity of factor VIII in blood coagulation. Inhibitory antibodies against the C2 domain commonly develop following factor VIII replacement therapy
Caileen M Brison+8 more
doaj +1 more source
Where Are the Baryons? III: Non-Equilibrium Effects and Observables [PDF]
Numerical simulations of the intergalactic medium have shown that at the present epoch a significant fraction (40-50%) of the baryonic component should be found in the (T~10^6K) Warm-Hot Intergalactic Medium (WHIM) - with several recent observational lines of evidence indicating the validity of the prediction.
arxiv +1 more source
Self-ionizing galactic winds [PDF]
We present hydrodynamical simulations of galactic winds from star-forming galaxies including non-equilibrium ionization and frequency-dependent radiative transfer, processes that have remained largely unaccounted for in galactic wind studies. We consider radiation from massive stars, the metagalactic UV/X-ray background, and the self-radiation of the ...
arxiv +1 more source
The factor VIII protein and its function.
Factor VIII (FVIII), an essential blood coagulation protein, is a key component of the fluid phase blood coagulation system. Human factor VIII is a single chain of about 300 kDa consisting of domains described as A1-A2-B-A3-C1-C2.
A. Mazurkiewicz-Pisarek+3 more
semanticscholar +1 more source
Recombinant factor VIIa concentrate versus plasma-derived concentrates for treating acute bleeding episodes in people with haemophilia and inhibitors [PDF]
In people with haemophilia, therapeutic clotting agents might be recognised as a foreign protein and induce anti-factor VIII antibodies, known as 'inhibitors'.
D'AMICO, Roberto+4 more
core +1 more source