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| Primary information |
|---|
| ID | 15113 |
| Therapeutic ID | Th1567 |
| Protein Name | Coagulation Factor IX Human |
| Sequence | >Th1567_Coagulation_Factor_IX_Human
YNSGKLEEFVQGNLERECMEEKCSFEEAREVFENTERTTEFWKQYVDGDQCESNPCLNGGSCKDDINSYECWCPFGFEGKNCELDVTCNIKNGRCEQFCKNSADNKVVCSCTEGYRLAENQKSCEPAVPFPCGRVSVSQTSKLTRAEAVFPDVDYVNSTEAETILDNITQSTQSFNDFTRVVGGEDAKPGQFPWQVVLNGKVDAFCGGSIVNEKWIVTAAHCVETGVKITVVAGEHNIEETEHTEQKRNVIRIIPHHNYNAAINKYNHDIALLELDEPLVLNSYVTPICIADKEYTNIFLKFGSGYVSGWGRVFHKGRSALVLQYLRVPLVDRATCLRSTKFTIYNNMFCAGFHEGGRDSCQGDSGGPHVTEVEGTSFLTGIISWGEECAMKGKYGIYTKVSRYVNWIKEKTKLT
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| Molecular Weight | 46548.2 |
| Chemical Formula | C2041H3136N558O641S25 |
| Isoelectric Point | 5.2 |
| Hydrophobicity | -0.431 |
| Melting point | 54 °C |
| Half-life | 18.8 ± 5.4 hours. |
| Description | Factor IX (or Christmas factor) is one of the serine proteases of the coagulation system; it belongs to peptidase family S1. Deficiency of this protein causes hemophilia B. |
| Indication/Disease | Factor IX is used to treat Christmas disease. Factor IX deficiency is treated by injection factor IX produced from human plasma. Tranexamic acid may be of value in patients undergoing surgery who have inherited factor IX deficiency in order to reduce the perioperative risk of bleeding. |
| 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. |
| Mechanism of 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. |
| Toxicity | NA |
| Metabolism | NA |
| Absorption | NA |
| NA |
| Clearance | 8.62 ± 1.7. |
| Categories | Blood and Blood Forming Organs |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Coagulation factor X,Coagulation factor XI,Coagulation factor VII,Coagulation factor VIII,Prothrombin,Prolow-density lipoprotein receptor-related protein 1,Vitamin K-dependent gamma-carboxylase |
| Brand Name | Bebulin VH |
| Company | Baxter Laboratories |
| Brand Description | Baxter Laboratories |
| Prescribed For | Intravenous |
| Chemical Name | 300 [iU]/1mL |
| Formulation | BEBULIN is contraindicated in patients with Known history of hypersensitivity reactions to the product Known allergy to heparin Known history of heparin-induced thrombocytopenia |
| Physical Appearance | Shortness of breath. A heartbeat that does not feel normal. Chest pain or pressure or a fast heartbeat. Coughing up blood. Weakness on 1 side of the body, trouble speaking or thinking, change in balance, drooping on one side of the face, or blurred eyesight. Swelling, warmth, numbness, change of color, or pain in a leg or arm. Cough. Bleeding that is new or worse. A burning, numbness, or tingling feeling that is not normal. Restlessness. Upset stomach or throwing up. |
| Route of Administration | NA |
| Recommended Dosage | BEBULIN is indicated for the prevention and control of bleeding episodes in adult patients with hemophilia B (congenital Factor IX deficiency or Christmas disease). |
| Contraindication | NA |
| Side Effects | 2. Vapor Heating is described in: World Health Organization (WHO) Technical Report, Series No. 924, 2004, Annex 4, Guidelines on viral inactivation and removal, procedures intended to assure the viral safety, of human blood plasma products. |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |