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| Primary information |
|---|
| ID | 11763 |
| 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 |
| Company | Pfizer Canada Ulc |
| Brand Description | Pfizer Canada Ulc |
| Prescribed For | Intravenous |
| Chemical Name | 500 unit / vial |
| 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 | fever, injection site reactions (pain, redness, or swelling), chills, headache, flushing (warmth, redness, or tingly feeling under your skin), weakness, nausea, vomiting, or dizziness |
| Route of Administration | BeneFIX is used to treat or prevent bleeding in people with hemophilia B. This medication is not for treating people with hemophilia A factor VIII deficiency. BeneFIX may also be used for purposes not listed in this medication guide. Warnings Follow all directions on your medicine label and package.... |
| Recommended Dosage | BeneFIX is a prescription medicine used to treat the symptoms of Bleeding Episodes associated with Hemophilia B and as Routine Prophylaxis to Hemophilia B. BeneFIX may be used alone or with other medications. |
| Contraindication | NA |
| Side Effects | Coagulation factor IX is the active ingredient in BeneFIX. 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. |
| Useful Link 1 | Link |
| Useful Link 2 | Link |
| Remarks | NA |