• 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)
  • Hemophilia A is an X-linked, recessive disorder caused by deficiency of functional plasma clotting factor VIII (FVIII), which may be inherited or arise from spontaneous mutation. (medscape.com)
  • Activated factor IX (IXa), complexed with activated factor VIII (VIIIa) on a phospholipid membrane surface, activates factor X, and activity normally expressed on the surface of activated platelets. (medscape.com)
  • Acquired hemophilia A (AHA) is a rare bleeding disorder caused by an autoantibody to coagulation factor (F) VIII. (biomedcentral.com)
  • Acquired hemophilia (AH) is a rare bleeding disorder caused by the spontaneous development of autoantibodies against coagulation factors, most commonly factor (F) VIII (acquired hemophilia A, AHA). (seedmedicalpublishers.com)
  • The prothrombinase complex is the enzymatic complex responsible for timely thrombin formation at the place of vascular injury and is composed of the enzyme, factor Xa (fXa), the non-enzymatic cofactor factor Va (fVa), and the substrate prothrombin assembled on a lipid membrane in the presence of divalent metal ions. (csuohio.edu)
  • Evidence for the involvement of sFlt1 in the occurrence of PE was initially provided by an animal model in which gravid rats were infected with a recombinant adenovirus encoding sFlt1 and compared to animals infected with the empty control adenovirus. (hindawi.com)
  • ZMapp), anti-sense phosphorodiamidate morpholino oligomers (PMO AVI-6002), lipid nanoparticle small interfering RNA (LNP-siRNA: TKM-Ebola), and an EBOV glycoprotein-based vaccine using live-attenuated recombinant vesicular stomatitis virus (rVSV-EBOGP) or a chimpanzee adenovirus (rChAd-EBOGP)-based vector. (biomedcentral.com)
  • Either activated factor XI (XIa) or the activated factor VII (VIIa)-tissue factor complex may activate factor IX. (medscape.com)
  • The live recombinant vaccine has shown effectiveness of 97.5% in preventing infection among 90,000 individuals in an active Ebola virus outbreak in the Democratic Republic of Congo. (medscape.com)
  • [ 1 ] but also, apparently, in vivo, and this may account for the finding that when factor IX is infused into hemophilia B patients, recovery is only 50% of what was expected. (medscape.com)
  • Acquired hemophilia (AH) is an autoimmune disease caused by the formation of inhibitory autoantibodies against coagulation factors. (seedmedicalpublishers.com)
  • These inhibitory autoantibodies (immunoglobulin IgG) neutralize the activation or function or accelerate the clearance of a specific clotting factor [1], mainly FVIII (acquired hemophilia A-AHA), although all the coagulation factors can be involved. (seedmedicalpublishers.com)
  • Coagulation factor IX is on the World Health Organization's List of Essential Medicines. (wikipedia.org)
  • 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)
  • 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)
  • 3. Kalafatis, M., Takahashi, Y., Girma, J.P., and Meyer, D. Localization of a collagen interactive domain of human von Willebrand Factor between amino acid residues Gly 911 and Glu 1365. (csuohio.edu)
  • Activated microthrombotic pathway mediates platelet activation and exocytosis of unusually large von Willebrand factor multimers (ULVWF) from ECs and initiates microthrombogenesis. (biomedcentral.com)
  • 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)
  • Factor IX is inhibited by antithrombin. (wikipedia.org)
  • Antithrombin (AT) is the primary inhibitor of factor IXa. (medscape.com)
  • Testing for inhibitors is indicated when bleeding is not controlled after infusion of adequate amounts of factor concentrate during a bleeding episode. (medscape.com)
  • PCCs may also contain small amounts of factors VIIa, IXa, and Xa. (medscape.com)
  • Products available on the market include prothrombin complex concentrates (PCCs), which contain prothrombin, factors VII and X, and proteins C and S in addition to factor IX. (medscape.com)
  • It is converted to thrombin by a prothrombin activator complex consisting of factor Xa, factor V, phospholipid, and calcium ions. (lookformedical.com)
  • Apart from CSCs, there are a myriad of factors and interactions between various cell types and the tumor microenvironment (TME) that ultimately affect CCA progression. (springer.com)
  • 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)
  • Platelets express a receptor/binding protein for factor IXa that promotes assembly of the IXa/VIIIa complex. (medscape.com)
  • 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)
  • Physical therapy guided by a therapist who is experienced in working with patients with connective-tissue abnormalities and joint dysfunction can be very helpful in the management of long-term health. (medscape.com)
  • Factor V accomplishes this by forming a complex with factor Xa, phospholipid, and calcium (prothrombinase complex). (lookformedical.com)
  • 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 coagulation system leans on a delicate balance between coagulant and anticoagulant factors. (csuohio.edu)
  • and FACTOR X. It is used for monitoring anticoagulant therapy with COUMARINS. (lookformedical.com)
  • An anticoagulant that acts by inhibiting the synthesis of vitamin K-dependent coagulation factors. (lookformedical.com)
  • 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)
  • The FDA approved atoltivimab/maftivimab/odesivimab (Inmazeb), a recombinant human monoclonal antibody combination. (medscape.com)
  • Some highly purified factor IX products are derived from human plasma. (medscape.com)
  • Trauma is defined as tissue damage characterized by structural changes and physiological disorders due to mechanical, thermal, electrical and chemical energies, ionized or nuclear radiation or absence of essential elements of life such as oxygen and heat. (eurarchmedres.org)
  • Like all viruses, the EBOV largely relies on host cell factors and physiological processes for its entry, replication, and egress. (biomedcentral.com)
  • there is some evidence that factor IX mutants lacking collagen-IV binding may exhibit greater recovery but may be associated with a mild bleeding tendency. (medscape.com)
  • We have identified the molecular defect in factor V Leiden and we have delineated specific amino acid regions on the factor V/Va molecule that are crucial for their functions. (csuohio.edu)
  • 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)
  • In factor IX-deficient patients with liver dysfunction, inability of the diseased liver to clear the activated factors may lead to thrombosis. (medscape.com)
  • Biochemical studies on collagen molecules are possible with cultured skin fibroblasts to confirm vascular-type EDS (type IV), arthrochalasia-type EDS (types VIIA and VIIB), and dermatosparaxis-type EDS (type VIIC). (medscape.com)
  • Traditionally, CSCs are identified through a combination of surface markers, however, there is a lack of consensus among the scientific community, especially as these combinations appear to be specific to the corresponding tissues and tumors. (springer.com)
  • Angiogenic factors and their receptors are important regulators of placental vascular development [ 13 ]. (hindawi.com)
  • The most widely studied serum markers for PE, to date, are vascular endothelial growth factor (VEGF) and placental growth factor (PlGF). (hindawi.com)
  • Similarly, sEng is a truncated form of receptor for two subtypes of transforming growth factor beta (TGF β ) specifically, TGF β 1 and TGF β 2 which are highly expressed by vascular endothelial cells and syncytiotrophoblasts. (hindawi.com)
  • The etiology is probably multifactorial: genetic and environmental factors interact in order to cause a loss of the immune tolerance and the formation of the inhibitory antibodies [2]. (seedmedicalpublishers.com)
  • Factor IX expression increases with age in humans and mice. (wikipedia.org)
  • Factor IX (plasma thromboplastin component [PTC]) is produced in the liver. (medscape.com)
  • Although factor IX products synthesized via recombinant DNA technology prevent transmission of prion disease, they are generally associated with lower intravascular recovery of factor IX than purified factor IX products prepared from plasma are. (medscape.com)
  • Clotting time of PLASMA recalcified in the presence of excess TISSUE THROMBOPLASTIN. (lookformedical.com)
  • This in turn results in an exponential production of multiple factors such as cytokines and growth factors leading to the clinical manifestations of PE [ 11 ]. (hindawi.com)
  • The clinical manifestation of AHA includes mostly spontaneous hemorrhages into skin, mucous membranes, muscles, soft tissues, or joints. (seedmedicalpublishers.com)
  • Factor IX deficiency is treated by injection of purified factor IX produced through cloning in various animal or animal cell vectors. (wikipedia.org)
  • We have recently demonstrated convincingly in melanoma and triple negative breast cancer cell lines that recombinant (r) hTRAIL together with several natural compounds (Quercetin, Silibinin, Ursolic Acid) are efficient in inducing apoptosis in previously described TRAIL-resistant cancer cell lines. (csuohio.edu)
  • 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)
  • Recombinant factor VIIa has been used to help control surgical bleeding, but experience is limited and the usual surgical precautions for patients with EDS should be followed. (medscape.com)
  • It is characterized by soft tissue bleeding in patients without a personal or family history of bleeding. (biomedcentral.com)
  • [ 9 ] They may be less expensive than purified factor IX products. (medscape.com)
  • [ 10 ] Recombinant factor IX products appear not to give rise to thrombosis. (medscape.com)
  • 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)
  • Cutaneous polyarteritis nodosa (CPN) is an uncommon form of vasculitis of the small-and medium-sized arteries in the reticular dermis and subcutaneous tissue. (annals.edu.sg)
  • Factor IX complex is on the WHO Model List of Essential Medicines , the most important medications needed in a basic health system . (wikidoc.org)
  • 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)
  • [13] Recombinant factor IX is used to treat Christmas disease. (wikidoc.org)
  • The reporter responds to the activity of stemness master transcriptional factors SOX2 and OCT4 through six concatenated repeats of a SOX2/OCT4 response element (SORE6) [ 14 ]. (springer.com)
  • Elevated factor IX concentrations are linked with an increased risk of thromboembolism. (medscape.com)
  • As a general rule, if PCCs are to be used for replacement therapy, factor IX levels should not exceed 50% of normal. (medscape.com)
  • We propose a therapeutic regimen to supplement the current supportive therapy aiming to reduce viral load, the most important factor in the determination of mortality. (biomedcentral.com)
  • [ 8 ] and is treated with factor IX replacement. (medscape.com)
  • Like sFlt1, soluble endoglin (sEng) is an antiangiogenic factor capable of inhibiting capillary tube formation in vitro [ 17 ]. (hindawi.com)
  • In mouse models, mutations within the promoter region of factor IX have an age-dependent phenotype. (wikipedia.org)
  • A list of all the mutations in Factor IX is compiled and maintained by EAHAD. (wikipedia.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)