CofactorBlood coagulationPathwayRecombinantHemophiliaCoagulation factorsMutations of factorInhibited by antithrombinActivateDeficiency of factorIntracranial hemorrhageWillebrandResiduesProteinGene for factorActivatorProthrombinaseProteinsVitaminPresencePlasmaListPatientsCirculatesBindsPathway inhibitorSerine proteasesMembraneCoagulationInhibitorsVIIIaPlateletsProcoagulantActivatesDeficiencyActivationComplexFormActivity
Cofactor3
- When activated into factor IXa, in the presence of Ca2+, membrane phospholipids, and a Factor VIII cofactor, it hydrolyses one arginine-isoleucine bond in factor X to form factor Xa. (wikipedia.org)
- Once bound to its cofactor, factor VII can be activated by a number of different proteases. (medscape.com)
- It serves as a cofactor with factor VIIa to activate factor X in the extrinsic pathway of blood coagulation. (lookformedical.com)
Blood coagulation5
- Factors VII, IX, and X all play key roles in blood coagulation and also share a common domain architecture. (wikipedia.org)
- It is a test of the intrinsic pathway (factors VIII, IX, XI, and XII) and the common pathway (fibrinogen, prothrombin, factors V and X) of BLOOD COAGULATION. (lookformedical.com)
- The process of the interaction of BLOOD COAGULATION FACTORS that results in an insoluble FIBRIN clot. (lookformedical.com)
- Activated form of factor X that participates in both the intrinsic and extrinsic pathways of blood coagulation. (lookformedical.com)
- Storage-stable glycoprotein blood coagulation factor that can be activated to factor Xa by both the intrinsic and extrinsic pathways. (lookformedical.com)
Pathway3
- It is processed to remove the signal peptide, glycosylated and then cleaved by factor XIa (of the contact pathway) or factor VIIa (of the tissue factor pathway) to produce a two-chain form, where the chains are linked by a disulfide bridge. (wikipedia.org)
- [ 4 ] The factor VIIa/TF complex activates both factors IX and X. It is inhibited by tissue factor pathway inhibitor (TFPI) in complex with factor Xa. (medscape.com)
- The hemostatic pathway: role of factor IX. (medscape.com)
Recombinant5
- The original Christmas disease mutation was identified by sequencing of Christmas' DNA, revealing a mutation which changed a cysteine to a serine.Recombinant factor IX is used to treat Christmas disease. (wikipedia.org)
- The Food and Drug Administration (FDA) approved recombinant activated factor VIII (synthetic vitamin K -dependent protein) for treatment of bleeding in patients with hemophilia A or B who have developed inhibitors against replacement coagulation factors. (medscape.com)
- Recombinant activated factor VII (rFVIIa) binds to exposed tissue factor at the place of tissue and vascular injury. (medscape.com)
- [13] Recombinant factor IX is used to treat Christmas disease. (wikidoc.org)
- tPA produced by such means are referred to as recombinant tissue plasminogen activator ( rtPA ). (wikidoc.org)
Hemophilia1
- Hemophilia B, or Christmas disease, is an inherited, recessive disorder that involves deficiency of functional coagulation factor IX (FIX) in plasma. (medscape.com)
Coagulation factors1
- An anticoagulant that acts by inhibiting the synthesis of vitamin K-dependent coagulation factors. (lookformedical.com)
Mutations of factor2
- Formulations include: nonacog alfa (brand name BeneFix) albutrepenonacog alfa (brand name Idelvion) eftrenonacog alfa (brand name Alprolix) nonacog beta pegol (brand name Refixia) Some rare mutations of factor IX result in elevated clotting activity, and can result in clotting diseases, such as deep vein thrombosis. (wikipedia.org)
- Some rare mutations of factor IX result in elevated clotting activity, and can result in clotting diseases, such as deep vein thrombosis . (wikidoc.org)
Inhibited by antithrombin1
- Factor IX is inhibited by antithrombin. (wikipedia.org)
Activate1
- Carboxylation by vitamin K is necessary to activate this factor. (medscape.com)
Deficiency of factor2
- Deficiency of factor V leads to Owren's disease. (lookformedical.com)
- A deficiency of factor X, sometimes called Stuart-Prower factor deficiency, may lead to a systemic coagulation disorder. (lookformedical.com)
Intracranial hemorrhage1
- Nevertheless, factor VII-deficient newborns sometimes develop fatal intra-abdominal or intracranial hemorrhage. (medscape.com)
Willebrand1
- The process of thrombus formation is considered to be initiated by von Willebrand factor (VWF) binding to exposed collagen or laminin in the damaged vessel wall, followed by shear-dependent platelet binding to VWF through the glycoprotein (GP)Ib-V-IX complex ( 1 , 3 , 4 ). (frontiersin.org)
Residues2
- conclude that residues 88 to 109 of the second EGF domain mediate binding to platelets and assembly of the factor X activating complex. (wikipedia.org)
- These amino acid residues form much of the connective tissues in meat). (bionity.com)
Protein4
- Deficiency of this protein causes haemophilia B. It was discovered in 1952 after a young boy named Stephen Christmas was found to be lacking this exact factor, leading to haemophilia. (wikipedia.org)
- The factor IX protein is composed of four protein domains: the Gla domain, two tandem copies of the EGF domain and a C-terminal trypsin-like peptidase domain which carries out the catalytic cleavage. (wikipedia.org)
- Tissue plasminogen activator (abbreviated tPA or PLAT ) is a protein involved in the breakdown of blood clots . (wikidoc.org)
- Constituent composed of protein and phospholipid that is widely distributed in many tissues. (lookformedical.com)
Gene for factor3
- The gene for factor IX is located on the X chromosome (Xq27.1-q27.2) and is therefore X-linked recessive: mutations in this gene affect males much more frequently than females. (wikipedia.org)
- Polly, a transgenic cloned Poll Dorset sheep carrying the gene for factor IX, was produced by Dr Ian Wilmut at the Roslin Institute in 1997. (wikipedia.org)
- [ 1 ] The human factor VII gene is located on chromosome 13, very close to the gene for factor X. (medscape.com)
Activator2
- The physiologic activator of factor VII is thought to be factor Xa, although significant auto-activation by factor VIIa can occur. (medscape.com)
- It is converted to thrombin by a prothrombin activator complex consisting of factor Xa, factor V, phospholipid, and calcium ions. (lookformedical.com)
Prothrombinase2
- Phosphatidylserine-exposing platelets, usually located around a thrombus, provide a negatively charged membrane surface, which supports coagulation factor binding and the formation of tenase and prothrombinase complexes ( 6 , 7 ). (frontiersin.org)
- Factor V accomplishes this by forming a complex with factor Xa, phospholipid, and calcium (prothrombinase complex). (lookformedical.com)
Proteins1
- Several structures of 'super active' mutants have been solved, which reveal the nature of factor IX activation by other proteins in the clotting cascade. (wikipedia.org)
Vitamin1
- Its shortest half-life of all the clotting factors is reflected in the initial rapid prolongation of prothrombin time (INR) in therapy with vitamin K antagonists. (medscape.com)
Presence1
- Clotting time of PLASMA recalcified in the presence of excess TISSUE THROMBOPLASTIN. (lookformedical.com)
Plasma1
- Older patients who received unpurified plasma‐derived clotting factor concentrates may have signs and symptoms of infectious disease (eg, hepatitis, HIV infection). (medscape.com)
List4
- Coagulation factor IX is on the World Health Organization's List of Essential Medicines. (wikipedia.org)
- A list of all the mutations in Factor IX is compiled and maintained by EAHAD. (wikipedia.org)
- Factor IX complex is on the WHO Model List of Essential Medicines , the most important medications needed in a basic health system . (wikidoc.org)
- A list of all the mutations in Factor IX is compiled and maintained at the Factor IX mutation database [19] maintained at the University College London. (wikidoc.org)
Patients1
- 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. (wikipedia.org)
Circulates2
- Factor VII circulates as a single-chain zymogen with a molecular weight of about 50,000 daltons. (medscape.com)
- When mechanical or chemical damage of the vascular wall occurs, subendothelial TF is expressed/exposed to blood flow and binds plasma factor VIIa, which circulates as an enzyme at a concentration of approximately 0.1 nM (1% of plasma factor VII) [ 4 ] and escapes the inhibition by serine proteases inhibitors because of its poor enzymatic qualities [ 1 , 5 ]. (hindawi.com)
Binds3
- Factor VII binds to tissue factor. (medscape.com)
- Recombinant activated factor VII (rFVIIa) binds to exposed tissue factor at the place of tissue and vascular injury. (medscape.com)
- With a vascular injury, TF becomes exposed to blood and binds plasma factor VIIa, and the resulting complex initiates a series of enzymatic reactions leading to clot formation and vascular sealing. (hindawi.com)
Pathway inhibitor2
- [ 6 ] The factor VIIa/TF complex activates both factors IX and X. It is inhibited by tissue factor pathway inhibitor (TFPI) in complex with factor Xa. (medscape.com)
- TF-triggered procoagulant response is also down-regulated by the stoichiometric inhibitors tissue factor pathway inhibitor (TFPI) and antithrombin (AT). (hindawi.com)
Serine proteases1
- Factor IX and factor X are converted to the serine proteases factor IXa and factor Xa, which are the enzymatic components of the intrinsic factor Xase and the prothrombinase complexes, respectively. (hindawi.com)
Membrane1
- Tissue factor (TF) is an integral membrane protein that is essential to life. (hindawi.com)
Coagulation2
- Coagulation factor IX is on the World Health Organization's List of Essential Medicines. (wikipedia.org)
- TFPI serves to attenuate the activity of the extrinsic factor Xase, the trigger of coagulation. (hindawi.com)
Inhibitors1
- The components of these multistep processes are illustrated as follows: enzymes (pink circles), inhibitors (blue circles), zymogens (green boxes), or complexes (cream ovals). (hindawi.com)
VIIIa3
- The catalytic efficiency (kcat/Km) for factor X activation by factor VIIIa/factor IXa is far in excess of the catalytic efficiency of activation of factor X by tissue factor/factor VIIa (Table 3). (nih.gov)
- Factor IXa and factor Xa form complex enzymes with their nonenzymatic co-factors (factor VIIIa and factor Va, resp. (hindawi.com)
- The product of the protein Case reaction, activated protein C (APC), inactivates the cofactors factors Va and VIIIa. (hindawi.com)
Platelets1
- conclude that residues 88 to 109 of the second EGF domain mediate binding to platelets and assembly of the factor X activating complex. (wikipedia.org)
Procoagulant1
- It has the shortest half-life of the procoagulant factors, approximately 3-6 hours. (medscape.com)
Activates2
- Thrombin accelerates its own generation via several feed-back reactions, cleaves fibrinogen, and activates factor XIII, which leads to the formation of a crosslinked insoluble fibrin clot [ 6 , 7 ] (Figure 1 ). (hindawi.com)
- and activates factor XIII to form a cross-linked fibrin clot. (hindawi.com)
Deficiency5
- Deficiency of this protein causes haemophilia B. It was discovered in 1952 after a young boy named Stephen Christmas was found to be lacking this exact factor, leading to haemophilia. (wikipedia.org)
- Factor IX deficiency is treated by injection of purified factor IX produced through cloning in various animal or animal cell vectors. (wikipedia.org)
- 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. (wikipedia.org)
- In a survey, Peltier et al found that among 25 patients with congenital factor VII deficiency, initial bleeding symptoms included skin bruising (58%), epistaxis (56%), and menorrhagia (47% of reproductive-age females). (medscape.com)
- Factor VII testing is indicated when Factor VII deficiency is suspected. (medscape.com)
Activation2
- Several structures of 'super active' mutants have been solved, which reveal the nature of factor IX activation by other proteins in the clotting cascade. (wikipedia.org)
- The physiologic activator of factor VII is thought to be factor Xa, although significant auto-activation by factor VIIa can occur. (medscape.com)
Complex2
- It is a component of the factor VIIa-TF complex enzyme and plays a primary role in both normal hemostasis and thrombosis. (hindawi.com)
- TF is an integral transmembrane protein expressed by various cells, is a component of the factor VIIa-TF complex enzyme and is essential for normal hemostasis [ 1 , 2 ]. (hindawi.com)
Form1
- It is processed to remove the signal peptide, glycosylated and then cleaved by factor XIa (of the contact pathway) or factor VIIa (of the tissue factor pathway) to produce a two-chain form, where the chains are linked by a disulfide bridge. (wikipedia.org)
Activity1
- Formulations include: nonacog alfa (brand name BeneFix) albutrepenonacog alfa (brand name Idelvion) eftrenonacog alfa (brand name Alprolix) nonacog beta pegol (brand name Refixia) Some rare mutations of factor IX result in elevated clotting activity, and can result in clotting diseases, such as deep vein thrombosis. (wikipedia.org)