Anti-Factor IX/PTC antibody

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Factor IX also known as Christmas factor or PTC is an important clotting protein in the coagulation cascade. It is a 415 amino acid protein with a mass of approximately 55 kDa. Factor IX is mainly expressed in the liver and then released into the bloodstream where it plays a significant role in blood clotting processes. The recombinant version of this protein referred to as factor IX recombinant is used therapeutically especially in patients who require coagulation support due to factor deficiencies.

Biological function summary

The function of factor IX involves its role in the amplification phase of the coagulation cascade. It participates as part of the intrinsic tenase complex which forms on negatively charged phospholipids in the presence of calcium ions. This complex significantly enhances the conversion of factor X to its active form factor Xa an important step in the clotting process. The activity of factor IX can be assessed using assays such as the factor IX activity assay and ELISA allowing for precise measurement of its function and quantity.

Pathways

Factor IX is integral within the intrinsic pathway of blood coagulation. It interacts with proteins such as factor VIII and factor X. When activated factor IXa forms a complex with the cofactor factor VIIIa on membrane surfaces intensifying the transformation of factor X to factor Xa which then catalyzes the conversion of prothrombin to thrombin. This series of reactions leads to fibrin formation important for stable clot production. Factor IXa also relates to the pathways influencing cellular signaling and hemostasis.

Associated diseases and disorders

Factor IX deficiencies cause hemophilia B a genetic disorder that leads to improper blood clotting. This disorder is mainly characterized by spontaneous bleeding or severe bleeding after injury. Patients with hemophilia B usually have mutations in the factor IX gene on the X chromosome affecting the protein's function. Successful treatment often involves replacement therapy using factor IX concentrates or the recombinant form. The interaction with proteases such as factor VIIa highlights its importance in therapeutic strategies for bleeding disorders.