Human dysfibrinogenemia plasma

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Fibrinogen (Factor I) is a plasma soluble glycoprotein that is synthesized by the liver at a size of 340 kDa and circulating at a concentration of 2.6 to 3 mg/mL.
Fibrinogen is a dimer bound by disulfide bridges composed of 3 pairs of polypeptide chains not identical. Under the action of thrombin, fibrinogen is converted into fibrin. In combination with FXIII, calcium ions, fibrin forms a stable network that ensures coagulation.
Dysibrinogenemic plasma is plasma that has a functional fibrinogen defect.
Most of the patients are asymptomatic, the others may present with moderate hemorrhagic signs but also thromboses.