Hemophilia A is caused by defects in the factor VIII gene. This results in life-threatening hemorrhages and severe arthropathies. Today, hemophiliacs are treated with human blood-derived factor VIII. In the future, it may be possible to use gene therapy to avoid long-term complications of conventional therapy and to improve the quality of life. However, initial gene therapy models using retrovi...

It has been suggested that the basis for the marked discordance in factor VIII parameters in preeclampsia is the result of proteolysis of the factor VIII procoagulant component (VIII:C) owing to activation of the coagulation system. To investigate this further, levels were compared of factor VIII:C and VIII:C (Ag), the immunologic equivalent of the procoagulant activity, in a series of preeclam...

A recent review article in Blood’ dealt with the advantage of using highly purified factor VI11 (F VIII) concentrates for the treatment of hemophilia. These products have a high specific activity of F VIIIC and, if properly treated, do not contain contaminating viruses. However, despite a relatively high purity, therapeutic concentrations of conventionally prepared F VI11 concentrates still hav...

Factor VIII, a protein cofactor involved in blood coagulation, functions in vitro on a phospholipid membrane surface to greatly increase the rate of factor X activation by factor IXa. Using gel filtration, rapid sedimentation, and resonance energy transfer we have studied the interaction of recombinant-derived human factor VIII with small and large unilamellar phospholipid vesicles composed of ...

The possible role of Ca2+ as an essential constituent part of the human factor VIII complex has been investigated by stability studies, metal determinations, and gel filtration experiments. In citrated plasma, the factor VIII coagulant activity (VIII:C) deteriorated during storage in a biphasic manner. Collection of blood in heparin, instead of chelating anticoagulants, or neutralization of cit...

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Factor VII circulates as a single chain inactive zymogen (10 nmol/L) and a trace ( approximately 10-100 pmol/L) circulates as the 2-chain form, factor VIIa. Factor VII and factor VIIa were studied in a coagulation model using plasma concentrations of purified coagulation factors with reactions initiated with relipidated tissue factor (TF). Factor VII (10 nmol/L) extended the lag phase of thromb...

introduction: the prevalence of rare bleeding disorders, including combined factor v+viii deficiency are higher in iran than in developed countries. there are only a few reports which have been written concerning kidney transplantation in the patients suffering from these disorders. case report: a 22-year old girl, with a known case of combined factor v+viii deficiency, a history of bladder sto...