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15117 details
Primary information
ID15117
Therapeutic IDTh1567
Protein NameCoagulation Factor IX Human
Sequence>Th1567_Coagulation_Factor_IX_Human YNSGKLEEFVQGNLERECMEEKCSFEEAREVFENTERTTEFWKQYVDGDQCESNPCLNGGSCKDDINSYECWCPFGFEGKNCELDVTCNIKNGRCEQFCKNSADNKVVCSCTEGYRLAENQKSCEPAVPFPCGRVSVSQTSKLTRAEAVFPDVDYVNSTEAETILDNITQSTQSFNDFTRVVGGEDAKPGQFPWQVVLNGKVDAFCGGSIVNEKWIVTAAHCVETGVKITVVAGEHNIEETEHTEQKRNVIRIIPHHNYNAAINKYNHDIALLELDEPLVLNSYVTPICIADKEYTNIFLKFGSGYVSGWGRVFHKGRSALVLQYLRVPLVDRATCLRSTKFTIYNNMFCAGFHEGGRDSCQGDSGGPHVTEVEGTSFLTGIISWGEECAMKGKYGIYTKVSRYVNWIKEKTKLT
Molecular Weight46548.2
Chemical FormulaC2041H3136N558O641S25
Isoelectric Point5.2
Hydrophobicity-0.431
Melting point54 °C
Half-life18.8 ± 5.4 hours.
DescriptionFactor 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/DiseaseFactor 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.
PharmacodynamicsBinds 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 ActionCoagulation 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.
ToxicityNA
MetabolismNA
AbsorptionNA
NA
Clearance8.62 ± 1.7.
CategoriesEnzymes and Coenzymes
Patents NumberNA
Date of IssueNA
Date of ExpiryNA
Drug InteractionNA
TargetCoagulation 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 NameKcentra
CompanyCSL Behring GmbH
Brand DescriptionCSL Behring GmbH
Prescribed ForIntravenous
Chemical NameNA
FormulationKcentra is contraindicated in: Patients with known anaphylactic or severe systemic reactions to Kcentra or any components in Kcentra including heparin, Factors II, VII, IX, X, Proteins C and S, Antithrombin III and Human albumin. Patients with disseminated intravascular coagulation (DIC). Patients with known heparin-induced thrombocytopenia (HIT). Kcentra contains heparin [see DESCRIPTION].
Physical Appearance headache, nausea, vomiting, joint pain, low blood pressure (hypotension), and low levels of iron in the blood (anemia)
Route of AdministrationKcentra is a blood coagulation factor replacement product. Kcentra is used to quickly reverse the effects of a blood-thinning medicine (such as warfarin) during a major bleeding episode, or when there is a need for emergency surgery or invasive medical procedure. Kcentra is for use in adults and dosing...
Recommended DosageKcentra is a prescription medicine used to treat the symptoms of Vitamin K Antagonist Reversal. Kcentra may be used alone or with other medications.
ContraindicationNA
Side EffectsNA
Useful Link 1Link
Useful Link 2Link
RemarksNA