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| Primary information |
|---|
| ID | 11774 |
| Therapeutic ID | Th1256 |
| Protein Name | Coagulation Factor IX (Recombinant) |
| Sequence | >Th1256_Coagulation_Factor_IX_(Recombinant)
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 | Recombinant Coagulation Factor IX is a purified Factor IX glycoprotein produced by recombinant DNA technology. It has a primary amino acid sequence that is identical to the Ala148 allelic form of human factor IX, and has structural and functional characteristics similar to those of endogenous factor IX. It is not derived from human blood (unlike human Factor IX complex), and is instead produced by a genetically engineered Chinese hamster ovary (CHO) cell line that secretes recombinant Factor IX into cell medium that is then processed and purified for use as a pharmaceutical agent. Recombinant Factor IX is indicated for the control and prevention of bleeding episodes in adult and pediatric patients with congenital factor IX deficiency (Hemophilia B). |
| Indication/Disease | For treatment of hemophilia (Christmas disease). |
| 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 | NA |
| 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 | Benefix - (250iu) |
| Company | Wyeth Ltd. |
| Brand Description | Wyeth Ltd. |
| Prescribed For | Intravenous |
| Chemical Name | NA |
| Formulation | BeneFIX is contraindicated in patients who have manifested life-threatening, immediate hypersensitivity reactions, including anaphylaxis, to the product or its components, including hamster protein. |
| Physical Appearance | hives, difficulty breathing, swelling of your face, lips, tongue, or throat, itching, wheezing, tightness in your chest, fast heartbeats, blue lips, lightheadedness, weight gain, swelling in your waist, hands, or lower legs, loss of appetite, fever, chills, continued bleeding after treatment, new or worsening bleeding, sudden numbness or weakness (especially on one side of the body), slurred speech, problems with vision or balance, chest pain, coughing up blood, and pain, swelling, warmth and redness in one or both legs |
| Route of Administration | BeneFIX is a medicine that contains the active substance nonacog alfa. It is available as a powder and solvent that are mixed together to form a solution for injection. |
| Recommended Dosage | BeneFIX is used for the treatment and prevention of bleeding in adults and children with haemophilia B (an inherited bleeding disorder). BeneFIX is intended for either short-term or long-term use. |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |