Using Factor VII in Hemophilia Gene Therapy

Abstract

Human blood at physiological conditions is kept as fluid through precise system called homeostasis, if damage to the vessel, causing the system will be restored by vessel wall. Cases no regulation or homeostasis disorders, thrombosis (intravascular coagulation) or bleeding occur. In normal conditions, the secretion of vascular endothelial heparin-like and trmbomodulin molecules prevent blood coagulation and secretion of nitric oxide and prostacyclin prevent platelet aggregation and blood brings the liquid keeps. Homeostasis has three stages: vasoconstriction, platelet plug formation and blood coagulation, blood coagulation are reactions in which plasma zymogens become active enzymes that create the clotting reaction. Coagulation reactions will be set with inhibitory and stimulatory mechanisms. Coagulation is a regulatory process that keeps the blood flowing. Blood coagulation has two external and internal pathways (Figure 1), tissue factor and FVII form the external pathway, internal pathway is formed of FVIII, FIX and FXI (Ramanarayana et al., 2011; Ellison, 1977).