Coagulation Factor IX (Recombinant)

Identification

Name
Coagulation Factor IX (Recombinant)
Accession Number
DB00100 / 382L14738L
Groups
BeneFIX
Description

Recombinant Coagulation aspect IX is a purified issue IX glycoprotein produced by recombinant DNA era. It has a number one amino acid series this is same to the Ala148 allelic shape of human factor IX, and has structural and useful traits similar to those of endogenous issue IX. It is not derived from human blood (not like human factor IX complicated), and is alternatively produced by means of a genetically engineered chinese language hamster ovary (CHO) mobile line that secretes recombinant element IX into cellular medium this is then processed and purified to be used as a pharmaceutical agent. Recombinant factor IX is indicated for the manipulate and prevention of bleeding episodes in grownup and pediatric patients with congenital thing IX deficiency (Hemophilia B).

Pharmacology

Pharmacodynamics

Binds vitamin K and factor VIIIa. Cleaves the Arg-Ile bond in factor X to form active factor Xa. Plays a key role in blood coagulation and clotting. Injections of factor IX are used to treat hemophilia B, which is sometimes called Christmas disease. AlphaNine is injected to increase plasma levels of Factor IX and can temporarily correct this coagulation defect. The activated partial thromboplastin time (aPTT) is prolonged in people with hemophilia B. Treatment with factor IX concentrate may normalize the aPTT by temporarily replacing the factor IX. The administration of BeneFIX increases plasma levels of factor IX, and can temporarily correct the coagulation defect in these patients.

Indication

For treatment of hemophilia (Christmas disease).

Action

Coagulation Factor IX is an important protein in the process of hemostasis and normal blood clotting as it plays a key role within the coagulation cascade. It is located within the blood plasma as a zymogen, an antecedent to enzymatic function, in its inactivated state. Factor IX is dependent on the presence of Vitamin K, and is activated to a serine protease by the function of Coagulation Factor XIa. Factor XIa cleaves the peptide bond associated with protein activation in Factor IX, leaving Factor IX with two exposed chains, a light chain and a heavy chain. These two chains are held together by several disulfide bonds that reinforce the structure of Factor IX's activated form. After being activated, Factor IX forms a complex with calcium ions, membrane phospholipids and Coagulation Factor VIII to activate Coagulation Factor X. The activation of Factor X then performs a similarly integral step in the blood coagulation cascade. The ultimate result of phenotypically normal coagulation factors is the creation of platelets for normal blood clotting.