Activated Factor IX, also known as Factor IXa or FIXa, is a crucial component of the coagulation cascade, which is a complex series of enzymatic reactions involved in the formation of blood clots. It is a protease enzyme synthesized in the liver, and its normal function is to assist in the formation of a stable blood clot at the site of injury.
Activated Factor IX is produced when Factor IX interacts with its activating cofactor, Factor VIIIa, in the presence of calcium ions and negatively charged surfaces such as phospholipids. Once activated, Factor IXa plays a pivotal role in the clotting process by acting as an intermediate protease, cleaving and activating Factor X, another crucial clotting factor. This conversion of Factor X into its active form, Factor Xa, triggers a cascade of reactions that ultimately leads to the formation of fibrin, the protein network that stabilizes the blood clot.
Deficiencies or defects in Activated Factor IX can result in Hemophilia B, a genetic bleeding disorder characterized by prolonged bleeding, both spontaneous and after trauma or surgery. Hemophilia B patients typically exhibit insufficient levels or impaired function of Factor IX, leading to impaired clotting ability and an increased risk of bleeding episodes.
Therapeutically, replacement therapy with recombinant or plasma-derived Factor IX concentrates can be administered to individuals with Hemophilia B. The introduction of Activated Factor IX concentrate aims to restore the deficient or defective clotting factor, enabling effective clot formation and the prevention or management of bleeding episodes.
