Synthesis of Factor VIII by endothelial cells

  • Eric A. Jaffe
Part of the Developments in Cardiovascular Medicine book series (DICM, volume 27)


Factor VIII circulates in plasma in vivo as a high molecular weight (>1, 200 Kd) complex composed of two different glycoproteins. The major component (probably about 99% by weight) of the complex is called Factor VIII: von Willebrand Factor (VII:vWF) and is composed of 200–225 Kd disulfide-linked subunits. VIII: vWF can be measured antigenically as VIII: RAg using several different sensitive and specific immunoassays and is present in normal plasma at levels of about 10 μg/ml (1–5). VIII:vWF can also be measured functionally using several different types of assays including those involving the aggregation of platelets by the antibiotic ristocetin and those utilizing fixed platelets as a source of VIII: vWF receptor (5–7). VIII: RAg and VIII: vWF levels are both normal or increased in plasma from patients with hemophilia A and decreased in plasma from patients with classical von Willebrand’s disease (4–6). The other component of the complex is called antihemophilic factor (VIII: AHF or VIII: C) and is thought to have a molecular weight of about 285 Kd in the native state though present purifications yield a partially proteolyzed but functionally active product (5, 8–12). VIII: C can be measured antigenically as VIII: CAg by specific immunoassay (5, 13, 14) and is present in plasma at levels of <100 ng/ml. VIII: C can also be measured functionally using a specific clotting assay for procoagulant Factor VIII. VIII: CAg and VIII: C levels are reduced in plasma from most patients with hemophilia A and classical von Willebrand’s disease (5, 13, 14). While VIII: vWF and VIII: C circulate in plasma as a complex, they can be separated by a variety of procedures in vitro (5, 15, 16) and are not related antigenically (5, 13) (see reference 5 for a comprehensive recent review of the factor VIII complex).


Endothelial Cell Factor VIII Human Umbilical Vein Endothelial Cell Human Endothelial Cell Bovine Aortic Endothelial Cell 
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© Martinus Nijhoff Publishers, Boston 1984

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  • Eric A. Jaffe

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