Factor VIII: Blood-coagulation factor VIII. Antihemophilic factor that is part of the factor VIII/von Willebrand factor complex. Factor VIII is produced in the liver and acts in the intrinsic pathway of blood coagulation. It serves as a cofactor in factor X activation and this action is markedly enhanced by small amounts of thrombin.Blood Coagulation: The process of the interaction of BLOOD COAGULATION FACTORS that results in an insoluble FIBRIN clot.Blood Coagulation Factors: Endogenous substances, usually proteins, that are involved in the blood coagulation process.Factor IX: Storage-stable blood coagulation factor acting in the intrinsic pathway. Its activated form, IXa, forms a complex with factor VIII and calcium on platelet factor 3 to activate factor X to Xa. Deficiency of factor IX results in HEMOPHILIA B (Christmas Disease).Factor XIII: A fibrin-stabilizing plasma enzyme (TRANSGLUTAMINASES) that is activated by THROMBIN and CALCIUM to form FACTOR XIIIA. It is important for stabilizing the formation of the fibrin polymer (clot) which culminates the coagulation cascade.Hemophilia A: The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.Factor X: Storage-stable glycoprotein blood coagulation factor that can be activated to factor Xa by both the intrinsic and extrinsic pathways. A deficiency of factor X, sometimes called Stuart-Prower factor deficiency, may lead to a systemic coagulation disorder.Factor Xa: Activated form of factor X that participates in both the intrinsic and extrinsic pathways of blood coagulation. It catalyzes the conversion of prothrombin to thrombin in conjunction with other cofactors.Factor VII: Heat- and storage-stable plasma protein that is activated by tissue thromboplastin to form factor VIIa in the extrinsic pathway of blood coagulation. The activated form then catalyzes the activation of factor X to factor Xa.Blood Coagulation Tests: Laboratory tests for evaluating the individual's clotting mechanism.Factor XIa: Activated form of factor XI. In the intrinsic pathway, Factor XI is activated to XIa by factor XIIa in the presence of cofactor HMWK; (HIGH MOLECULAR WEIGHT KININOGEN). Factor XIa then activates factor IX to factor IXa in the presence of calcium.Thromboplastin: Constituent composed of protein and phospholipid that is widely distributed in many tissues. It serves as a cofactor with factor VIIa to activate factor X in the extrinsic pathway of blood coagulation.Factor IXa: Activated form of factor IX. This activation can take place via the intrinsic pathway by the action of factor XIa and calcium, or via the extrinsic pathway by the action of factor VIIa, thromboplastin, and calcium. Factor IXa serves to activate factor X to Xa by cleaving the arginyl-leucine peptide bond in factor X.Factor VIIa: Activated form of factor VII. Factor VIIa activates factor X in the extrinsic pathway of blood coagulation.Coagulants: Agents that cause clotting.Blood Coagulation Disorders: Hemorrhagic and thrombotic disorders that occur as a consequence of abnormalities in blood coagulation due to a variety of factors such as COAGULATION PROTEIN DISORDERS; BLOOD PLATELET DISORDERS; BLOOD PROTEIN DISORDERS or nutritional conditions.Factor V: Heat- and storage-labile plasma glycoprotein which accelerates the conversion of prothrombin to thrombin in blood coagulation. Factor V accomplishes this by forming a complex with factor Xa, phospholipid, and calcium (prothrombinase complex). Deficiency of factor V leads to Owren's disease.Factor XI: Stable blood coagulation factor involved in the intrinsic pathway. The activated form XIa activates factor IX to IXa. Deficiency of factor XI is often called hemophilia C.Thrombin: An enzyme formed from PROTHROMBIN that converts FIBRINOGEN to FIBRIN.Factor VIIIa: Activated form of factor VIII. The B-domain of factor VIII is proteolytically cleaved by thrombin to form factor VIIIa. Factor VIIIa exists as a non-covalent dimer in a metal-linked (probably calcium) complex and functions as a cofactor in the enzymatic activation of factor X by factor IXa. Factor VIIIa is similar in structure and generation to factor Va.Partial Thromboplastin Time: The time required for the appearance of FIBRIN strands following the mixing of PLASMA with phospholipid platelet substitute (e.g., crude cephalins, soybean phosphatides). 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. It is used as a screening test and to monitor HEPARIN therapy.Prothrombin: A plasma protein that is the inactive precursor of thrombin. It is converted to thrombin by a prothrombin activator complex consisting of factor Xa, factor V, phospholipid, and calcium ions. Deficiency of prothrombin leads to hypoprothrombinemia.Blood Coagulation Factor Inhibitors: Substances, usually endogenous, that act as inhibitors of blood coagulation. They may affect one or multiple enzymes throughout the process. As a group, they also inhibit enzymes involved in processes other than blood coagulation, such as those from the complement system, fibrinolytic enzyme system, blood cells, and bacteria.Prothrombin Time: Clotting time of PLASMA recalcified in the presence of excess TISSUE THROMBOPLASTIN. Factors measured are FIBRINOGEN; PROTHROMBIN; FACTOR V; FACTOR VII; and FACTOR X. It is used for monitoring anticoagulant therapy with COUMARINS.Disseminated Intravascular Coagulation: A disorder characterized by procoagulant substances entering the general circulation causing a systemic thrombotic process. The activation of the clotting mechanism may arise from any of a number of disorders. A majority of the patients manifest skin lesions, sometimes leading to PURPURA FULMINANS.Factor XII: Stable blood coagulation factor activated by contact with the subendothelial surface of an injured vessel. Along with prekallikrein, it serves as the contact factor that initiates the intrinsic pathway of blood coagulation. Kallikrein activates factor XII to XIIa. Deficiency of factor XII, also called the Hageman trait, leads to increased incidence of thromboembolic disease. Mutations in the gene for factor XII that appear to increase factor XII amidolytic activity are associated with HEREDITARY ANGIOEDEMA TYPE III.Hemophilia B: A deficiency of blood coagulation factor IX inherited as an X-linked disorder. (Also known as Christmas Disease, after the first patient studied in detail, not the holy day.) Historical and clinical features resemble those in classic hemophilia (HEMOPHILIA A), but patients present with fewer symptoms. Severity of bleeding is usually similar in members of a single family. Many patients are asymptomatic until the hemostatic system is stressed by surgery or trauma. Treatment is similar to that for hemophilia A. (From Cecil Textbook of Medicine, 19th ed, p1008)Protein C: A vitamin-K dependent zymogen present in the blood, which, upon activation by thrombin and thrombomodulin exerts anticoagulant properties by inactivating factors Va and VIIIa at the rate-limiting steps of thrombin formation.Factor Va: Activated form of factor V. It is an essential cofactor for the activation of prothrombin catalyzed by factor Xa.1-Carboxyglutamic Acid: Found in various tissues, particularly in four blood-clotting proteins including prothrombin, in kidney protein, in bone protein, and in the protein present in various ectopic calcifications.Vitamin K: A lipid cofactor that is required for normal blood clotting. Several forms of vitamin K have been identified: VITAMIN K 1 (phytomenadione) derived from plants, VITAMIN K 2 (menaquinone) from bacteria, and synthetic naphthoquinone provitamins, VITAMIN K 3 (menadione). Vitamin K 3 provitamins, after being alkylated in vivo, exhibit the antifibrinolytic activity of vitamin K. Green leafy vegetables, liver, cheese, butter, and egg yolk are good sources of vitamin K.Antithrombin III: A plasma alpha 2 glycoprotein that accounts for the major antithrombin activity of normal plasma and also inhibits several other enzymes. It is a member of the serpin superfamily.Fibrinogen: Plasma glycoprotein clotted by thrombin, composed of a dimer of three non-identical pairs of polypeptide chains (alpha, beta, gamma) held together by disulfide bonds. Fibrinogen clotting is a sol-gel change involving complex molecular arrangements: whereas fibrinogen is cleaved by thrombin to form polypeptides A and B, the proteolytic action of other enzymes yields different fibrinogen degradation products.Transglutaminases: Transglutaminases catalyze cross-linking of proteins at a GLUTAMINE in one chain with LYSINE in another chain. They include keratinocyte transglutaminase (TGM1 or TGK), tissue transglutaminase (TGM2 or TGC), plasma transglutaminase involved with coagulation (FACTOR XIII and FACTOR XIIIa), hair follicle transglutaminase, and prostate transglutaminase. Although structures differ, they share an active site (YGQCW) and strict CALCIUM dependence.von Willebrand Factor: A high-molecular-weight plasma protein, produced by endothelial cells and megakaryocytes, that is part of the factor VIII/von Willebrand factor complex. The von Willebrand factor has receptors for collagen, platelets, and ristocetin activity as well as the immunologically distinct antigenic determinants. It functions in adhesion of platelets to collagen and hemostatic plug formation. The prolonged bleeding time in VON WILLEBRAND DISEASES is due to the deficiency of this factor.Hemostasis: The process which spontaneously arrests the flow of BLOOD from vessels carrying blood under pressure. It is accomplished by contraction of the vessels, adhesion and aggregation of formed blood elements (eg. ERYTHROCYTE AGGREGATION), and the process of BLOOD COAGULATION.Arcidae: A family of ark shell mollusks, in the class BIVALVIA. They have soft bodies with platelike GILLS enclosed within two shells hinged together.Amino Acid Sequence: The order of amino acids as they occur in a polypeptide chain. This is referred to as the primary structure of proteins. It is of fundamental importance in determining PROTEIN CONFORMATION.Thrombelastography: Use of a thrombelastograph, which provides a continuous graphic record of the physical shape of a clot during fibrin formation and subsequent lysis.Antithrombins: Endogenous factors and drugs that directly inhibit the action of THROMBIN, usually by blocking its enzymatic activity. They are distinguished from INDIRECT THROMBIN INHIBITORS, such as HEPARIN, which act by enhancing the inhibitory effects of antithrombins.Molecular Sequence Data: Descriptions of specific amino acid, carbohydrate, or nucleotide sequences which have appeared in the published literature and/or are deposited in and maintained by databanks such as GENBANK, European Molecular Biology Laboratory (EMBL), National Biomedical Research Foundation (NBRF), or other sequence repositories.Fibrin: A protein derived from FIBRINOGEN in the presence of THROMBIN, which forms part of the blood clot.Anticoagulants: Agents that prevent clotting.Binding Sites: The parts of a macromolecule that directly participate in its specific combination with another molecule.Vitamin K Deficiency: A nutritional condition produced by a deficiency of VITAMIN K in the diet, characterized by an increased tendency to hemorrhage (HEMORRHAGIC DISORDERS). Such bleeding episodes may be particularly severe in newborn infants. (From Cecil Textbook of Medicine, 19th ed, p1182)Fibrinolysis: The natural enzymatic dissolution of FIBRIN.Recombinant Proteins: Proteins prepared by recombinant DNA technology.Benzamidines: Amidines substituted with a benzene group. Benzamidine and its derivatives are known as peptidase inhibitors.Kinetics: The rate dynamics in chemical or physical systems.Factor XII Deficiency: An absence or reduced level of blood coagulation factor XII. It normally occurs in the absence of patient or family history of hemorrhagic disorders and is marked by prolonged clotting time.Whole Blood Coagulation Time: The time required by whole blood to produce a visible clot.Blood Coagulation Disorders, Inherited: Hemorrhagic and thrombotic disorders that occur as a consequence of inherited abnormalities in blood coagulation.Factor XIIa: Activated form of factor XII. In the initial event in the intrinsic pathway of blood coagulation, kallikrein (with cofactor HIGH MOLECULAR WEIGHT KININOGEN) cleaves factor XII to XIIa. Factor XIIa is then further cleaved by kallikrein, plasmin, and trypsin to yield smaller factor XII fragments (Hageman-Factor fragments). These fragments increase the activity of prekallikrein to kallikrein but decrease the procoagulant activity of factor XII.Peptide Fragments: Partial proteins formed by partial hydrolysis of complete proteins or generated through PROTEIN ENGINEERING techniques.Protein S: The vitamin K-dependent cofactor of activated PROTEIN C. Together with protein C, it inhibits the action of factors VIIIa and Va. A deficiency in protein S; (PROTEIN S DEFICIENCY); can lead to recurrent venous and arterial thrombosis.Protein Binding: The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.von Willebrand Diseases: Group of hemorrhagic disorders in which the VON WILLEBRAND FACTOR is either quantitatively or qualitatively abnormal. They are usually inherited as an autosomal dominant trait though rare kindreds are autosomal recessive. Symptoms vary depending on severity and disease type but may include prolonged bleeding time, deficiency of factor VIII, and impaired platelet adhesion.Coagulation Protein Disorders: Hemorrhagic and thrombotic disorders resulting from abnormalities or deficiencies of coagulation proteins.Factor XI Deficiency: A hereditary deficiency of blood coagulation factor XI (also known as plasma thromboplastin antecedent or PTA or antihemophilic factor C) resulting in a systemic blood-clotting defect called hemophilia C or Rosenthal's syndrome, that may resemble classical hemophilia.Serine Endopeptidases: Any member of the group of ENDOPEPTIDASES containing at the active site a serine residue involved in catalysis.Thrombin Time: Clotting time of PLASMA mixed with a THROMBIN solution. It is a measure of the conversion of FIBRINOGEN to FIBRIN, which is prolonged by AFIBRINOGENEMIA, abnormal fibrinogen, or the presence of inhibitory substances, e.g., fibrin-fibrinogen degradation products, or HEPARIN. BATROXOBIN, a thrombin-like enzyme unaffected by the presence of heparin, may be used in place of thrombin.Blood Platelets: Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation.Serine Proteinase Inhibitors: Exogenous or endogenous compounds which inhibit SERINE ENDOPEPTIDASES.Factor V Deficiency: A deficiency of blood coagulation factor V (known as proaccelerin or accelerator globulin or labile factor) leading to a rare hemorrhagic tendency known as Owren's disease or parahemophilia. It varies greatly in severity. Factor V deficiency is an autosomal recessive trait. (Dorland, 27th ed)Factor XIII Deficiency: A deficiency of blood coagulation FACTOR XIII or fibrin stabilizing factor (FSF) that prevents blood clot formation and results in a clinical hemorrhagic diathesis.Thrombosis: Formation and development of a thrombus or blood clot in the blood vessel.Enzyme Precursors: Physiologically inactive substances that can be converted to active enzymes.Phospholipids: Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides see GLYCEROPHOSPHOLIPIDS) or sphingosine (SPHINGOLIPIDS). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system.Protein Conformation: The characteristic 3-dimensional shape of a protein, including the secondary, supersecondary (motifs), tertiary (domains) and quaternary structure of the peptide chain. PROTEIN STRUCTURE, QUATERNARY describes the conformation assumed by multimeric proteins (aggregates of more than one polypeptide chain).Base Sequence: The sequence of PURINES and PYRIMIDINES in nucleic acids and polynucleotides. It is also called nucleotide sequence.Fibrin Fibrinogen Degradation Products: Soluble protein fragments formed by the proteolytic action of plasmin on fibrin or fibrinogen. FDP and their complexes profoundly impair the hemostatic process and are a major cause of hemorrhage in intravascular coagulation and fibrinolysis.Molecular Weight: The sum of the weight of all the atoms in a molecule.Hemorrhagic Disorders: Spontaneous or near spontaneous bleeding caused by a defect in clotting mechanisms (BLOOD COAGULATION DISORDERS) or another abnormality causing a structural flaw in the blood vessels (HEMOSTATIC DISORDERS).Protein Structure, Tertiary: The level of protein structure in which combinations of secondary protein structures (alpha helices, beta sheets, loop regions, and motifs) pack together to form folded shapes called domains. Disulfide bridges between cysteines in two different parts of the polypeptide chain along with other interactions between the chains play a role in the formation and stabilization of tertiary structure. Small proteins usually consist of only one domain but larger proteins may contain a number of domains connected by segments of polypeptide chain which lack regular secondary structure.Models, Molecular: Models used experimentally or theoretically to study molecular shape, electronic properties, or interactions; includes analogous molecules, computer-generated graphics, and mechanical structures.Hydroxyethyl Starch Derivatives: Starches that have been chemically modified so that a percentage of OH groups are substituted with 2-hydroxyethyl ether groups.Cattle: Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor.Electrophoresis, Polyacrylamide Gel: Electrophoresis in which a polyacrylamide gel is used as the diffusion medium.Heparin: A highly acidic mucopolysaccharide formed of equal parts of sulfated D-glucosamine and D-glucuronic acid with sulfaminic bridges. The molecular weight ranges from six to twenty thousand. Heparin occurs in and is obtained from liver, lung, mast cells, etc., of vertebrates. Its function is unknown, but it is used to prevent blood clotting in vivo and vitro, in the form of many different salts.Plasma Substitutes: Any liquid used to replace blood plasma, usually a saline solution, often with serum albumins, dextrans or other preparations. These substances do not enhance the oxygen- carrying capacity of blood, but merely replace the volume. They are also used to treat dehydration.Hemorrhage: Bleeding or escape of blood from a vessel.Platelet Aggregation: The attachment of PLATELETS to one another. This clumping together can be induced by a number of agents (e.g., THROMBIN; COLLAGEN) and is part of the mechanism leading to the formation of a THROMBUS.Plasma: The residual portion of BLOOD that is left after removal of BLOOD CELLS by CENTRIFUGATION without prior BLOOD COAGULATION.Factor XIIIa: Activated form of FACTOR XIII, a transglutaminase, which stabilizes the formation of the fibrin polymer (clot) culminating the blood coagulation cascade.Factor X Deficiency: Blood coagulation disorder usually inherited as an autosomal recessive trait, though it can be acquired. It is characterized by defective activity in both the intrinsic and extrinsic pathways, impaired thromboplastin time, and impaired prothrombin consumption.Thrombophilia: A disorder of HEMOSTASIS in which there is a tendency for the occurrence of THROMBOSIS.Enzyme Activation: Conversion of an inactive form of an enzyme to one possessing metabolic activity. It includes 1, activation by ions (activators); 2, activation by cofactors (coenzymes); and 3, conversion of an enzyme precursor (proenzyme or zymogen) to an active enzyme.Hypoprothrombinemias: Absence or reduced levels of PROTHROMBIN in the blood.Kaolin: The most common mineral of a group of hydrated aluminum silicates, approximately H2Al2Si2O8-H2O. It is prepared for pharmaceutical and medicinal purposes by levigating with water to remove sand, etc. (From Merck Index, 11th ed) The name is derived from Kao-ling (Chinese: "high ridge"), the original site. (From Grant & Hackh's Chemical Dictionary, 5th ed)Bleeding Time: Duration of blood flow after skin puncture. This test is used as a measure of capillary and platelet function.Hemostatics: Agents acting to arrest the flow of blood. Absorbable hemostatics arrest bleeding either by the formation of an artificial clot or by providing a mechanical matrix that facilitates clotting when applied directly to the bleeding surface. These agents function more at the capillary level and are not effective at stemming arterial or venous bleeding under any significant intravascular pressure.Factor VII Deficiency: An autosomal recessive characteristic or a coagulation disorder acquired in association with VITAMIN K DEFICIENCY. FACTOR VII is a Vitamin K dependent glycoprotein essential to the extrinsic pathway of coagulation.Prekallikrein: A plasma protein which is the precursor of kallikrein. Plasma that is deficient in prekallikrein has been found to be abnormal in thromboplastin formation, kinin generation, evolution of a permeability globulin, and plasmin formation. The absence of prekallikrein in plasma leads to Fletcher factor deficiency, a congenital disease.Phosphatidylserines: Derivatives of phosphatidic acids in which the phosphoric acid is bound in ester linkage to a serine moiety. Complete hydrolysis yields 1 mole of glycerol, phosphoric acid and serine and 2 moles of fatty acids.Thrombomodulin: A cell surface glycoprotein of endothelial cells that binds thrombin and serves as a cofactor in the activation of protein C and its regulation of blood coagulation.Macromolecular Substances: Compounds and molecular complexes that consist of very large numbers of atoms and are generally over 500 kDa in size. In biological systems macromolecular substances usually can be visualized using ELECTRON MICROSCOPY and are distinguished from ORGANELLES by the lack of a membrane structure.Sequence Homology, Amino Acid: The degree of similarity between sequences of amino acids. This information is useful for the analyzing genetic relatedness of proteins and species.Platelet Activation: A series of progressive, overlapping events, triggered by exposure of the PLATELETS to subendothelial tissue. These events include shape change, adhesiveness, aggregation, and release reactions. When carried through to completion, these events lead to the formation of a stable hemostatic plug.Mutation: Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.Genetic Vectors: DNA molecules capable of autonomous replication within a host cell and into which other DNA sequences can be inserted and thus amplified. Many are derived from PLASMIDS; BACTERIOPHAGES; or VIRUSES. They are used for transporting foreign genes into recipient cells. Genetic vectors possess a functional replicator site and contain GENETIC MARKERS to facilitate their selective recognition.Amino Acids: Organic compounds that generally contain an amino (-NH2) and a carboxyl (-COOH) group. Twenty alpha-amino acids are the subunits which are polymerized to form proteins.Calcium: A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.Fibrinopeptide A: Two small peptide chains removed from the N-terminal segment of the alpha chains of fibrinogen by the action of thrombin during the blood coagulation process. Each peptide chain contains 18 amino acid residues. In vivo, fibrinopeptide A is used as a marker to determine the rate of conversion of fibrinogen to fibrin by thrombin.Crystallography, X-Ray: The study of crystal structure using X-RAY DIFFRACTION techniques. (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)Antigens: Substances that are recognized by the immune system and induce an immune reaction.Afibrinogenemia: A deficiency or absence of FIBRINOGEN in the blood.Liver: A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.Collagen Type VIII: A non-fibrillar collagen originally found in DESCEMET MEMBRANE. It is expressed in endothelial cell layers and in tissues undergoing active remodeling. It is heterotrimer comprised of alpha1(VIII) and alpha2(VIII) chains.Carbon-Carbon Ligases: Enzymes that catalyze the joining of two molecules by the formation of a carbon-carbon bond. These are the carboxylating enzymes and are mostly biotinyl-proteins. EC 6.4.Isoantibodies: Antibodies from an individual that react with ISOANTIGENS of another individual of the same species.Peptides: Members of the class of compounds composed of AMINO ACIDS joined together by peptide bonds between adjacent amino acids into linear, branched or cyclical structures. OLIGOPEPTIDES are composed of approximately 2-12 amino acids. Polypeptides are composed of approximately 13 or more amino acids. PROTEINS are linear polypeptides that are normally synthesized on RIBOSOMES.Cell Line: Established cell cultures that have the potential to propagate indefinitely.Hemarthrosis: Bleeding into the joints. It may arise from trauma or spontaneously in patients with hemophilia.Reptilian Proteins: Proteins obtained from species of REPTILES.alpha-2-Antiplasmin: A member of the serpin superfamily found in plasma that inhibits the lysis of fibrin clots which are induced by plasminogen activator. It is a glycoprotein, molecular weight approximately 70,000 that migrates in the alpha 2 region in immunoelectrophoresis. It is the principal plasmin inactivator in blood, rapidly forming a very stable complex with plasmin.Argon Plasma Coagulation: A method of tissue ablation and bleeding control that uses ARGON plasma (ionized argon gas) to deliver a current of thermocoagulating energy to the area of tissue to be coagulated.Platelet Adhesiveness: The process whereby PLATELETS adhere to something other than platelets, e.g., COLLAGEN; BASEMENT MEMBRANE; MICROFIBRILS; or other "foreign" surfaces.Venous Thrombosis: The formation or presence of a blood clot (THROMBUS) within a vein.Platelet Count: The number of PLATELETS per unit volume in a sample of venous BLOOD.Time Factors: Elements of limited time intervals, contributing to particular results or situations.Immunoelectrophoresis, Two-Dimensional: Immunoelectrophoresis in which a second electrophoretic transport is performed on the initially separated antigen fragments into an antibody-containing medium in a direction perpendicular to the first electrophoresis.Epidermal Growth Factor: A 6-kDa polypeptide growth factor initially discovered in mouse submaxillary glands. Human epidermal growth factor was originally isolated from urine based on its ability to inhibit gastric secretion and called urogastrone. Epidermal growth factor exerts a wide variety of biological effects including the promotion of proliferation and differentiation of mesenchymal and EPITHELIAL CELLS. It is synthesized as a transmembrane protein which can be cleaved to release a soluble active form.Surface Plasmon Resonance: A biosensing technique in which biomolecules capable of binding to specific analytes or ligands are first immobilized on one side of a metallic film. Light is then focused on the opposite side of the film to excite the surface plasmons, that is, the oscillations of free electrons propagating along the film's surface. The refractive index of light reflecting off this surface is measured. When the immobilized biomolecules are bound by their ligands, an alteration in surface plasmons on the opposite side of the film is created which is directly proportional to the change in bound, or adsorbed, mass. Binding is measured by changes in the refractive index. The technique is used to study biomolecular interactions, such as antigen-antibody binding.Antibodies: Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).Kallikreins: Proteolytic enzymes from the serine endopeptidase family found in normal blood and urine. Specifically, Kallikreins are potent vasodilators and hypotensives and increase vascular permeability and affect smooth muscle. They act as infertility agents in men. Three forms are recognized, PLASMA KALLIKREIN (EC 3.4.21.34), TISSUE KALLIKREIN (EC 3.4.21.35), and PROSTATE-SPECIFIC ANTIGEN (EC 3.4.21.77).Ristocetin: An antibiotic mixture of two components, A and B, obtained from Nocardia lurida (or the same substance produced by any other means). It is no longer used clinically because of its toxicity. It causes platelet agglutination and blood coagulation and is used to assay those functions in vitro.Kininogens: Endogenous peptides present in most body fluids. Certain enzymes convert them to active KININS which are involved in inflammation, blood clotting, complement reactions, etc. Kininogens belong to the cystatin superfamily. They are cysteine proteinase inhibitors. HIGH-MOLECULAR-WEIGHT KININOGEN; (HMWK); is split by plasma kallikrein to produce BRADYKININ. LOW-MOLECULAR-WEIGHT KININOGEN; (LMWK); is split by tissue kallikrein to produce KALLIDIN.Crotalid Venoms: Venoms from snakes of the subfamily Crotalinae or pit vipers, found mostly in the Americas. They include the rattlesnake, cottonmouth, fer-de-lance, bushmaster, and American copperhead. Their venoms contain nontoxic proteins, cardio-, hemo-, cyto-, and neurotoxins, and many enzymes, especially phospholipases A. Many of the toxins have been characterized.Deamino Arginine Vasopressin: A synthetic analog of the pituitary hormone, ARGININE VASOPRESSIN. Its action is mediated by the VASOPRESSIN receptor V2. It has prolonged antidiuretic activity, but little pressor effects. It also modulates levels of circulating FACTOR VIII and VON WILLEBRAND FACTOR.Lipoproteins: Lipid-protein complexes involved in the transportation and metabolism of lipids in the body. They are spherical particles consisting of a hydrophobic core of TRIGLYCERIDES and CHOLESTEROL ESTERS surrounded by a layer of hydrophilic free CHOLESTEROL; PHOSPHOLIPIDS; and APOLIPOPROTEINS. Lipoproteins are classified by their varying buoyant density and sizes.Hirudins: Single-chain polypeptides of about 65 amino acids (7 kDa) from LEECHES that have a neutral hydrophobic N terminus, an acidic hydrophilic C terminus, and a compact, hydrophobic core region. Recombinant hirudins lack tyr-63 sulfation and are referred to as 'desulfato-hirudins'. They form a stable non-covalent complex with ALPHA-THROMBIN, thereby abolishing its ability to cleave FIBRINOGEN.Activated Protein C Resistance: A hemostatic disorder characterized by a poor anticoagulant response to activated protein C (APC). The activated form of Factor V (Factor Va) is more slowly degraded by activated protein C. Factor V Leiden mutation (R506Q) is the most common cause of APC resistance.Receptor, PAR-1: A thrombin receptor subtype that couples to HETEROTRIMERIC GTP-BINDING PROTEINS resulting in the activation of a variety of signaling mechanisms including decreased intracellular CYCLIC AMP, increased TYPE C PHOSPHOLIPASES and increased PHOSPHOLIPASE A2.Blood Transfusion: The introduction of whole blood or blood component directly into the blood stream. (Dorland, 27th ed)Antibodies, Monoclonal: Antibodies produced by a single clone of cells.Cricetinae: A subfamily in the family MURIDAE, comprising the hamsters. Four of the more common genera are Cricetus, CRICETULUS; MESOCRICETUS; and PHODOPUS.Blood Proteins: Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.Snake Venoms: Solutions or mixtures of toxic and nontoxic substances elaborated by snake (Ophidia) salivary glands for the purpose of killing prey or disabling predators and delivered by grooved or hollow fangs. They usually contain enzymes, toxins, and other factors.Receptors, Thrombin: A family of proteinase-activated receptors that are specific for THROMBIN. They are found primarily on PLATELETS and on ENDOTHELIAL CELLS. Activation of thrombin receptors occurs through the proteolytic action of THROMBIN, which cleaves the N-terminal peptide from the receptor to reveal a new N-terminal peptide that is a cryptic ligand for the receptor. The receptors signal through HETEROTRIMERIC GTP-BINDING PROTEINS. Small synthetic peptides that contain the unmasked N-terminal peptide sequence can also activate the receptor in the absence of proteolytic activity.Binding, Competitive: The interaction of two or more substrates or ligands with the same binding site. The displacement of one by the other is used in quantitative and selective affinity measurements.Warfarin: An anticoagulant that acts by inhibiting the synthesis of vitamin K-dependent coagulation factors. Warfarin is indicated for the prophylaxis and/or treatment of venous thrombosis and its extension, pulmonary embolism, and atrial fibrillation with embolization. It is also used as an adjunct in the prophylaxis of systemic embolism after myocardial infarction. Warfarin is also used as a rodenticide.Antifibrinolytic Agents: Agents that prevent fibrinolysis or lysis of a blood clot or thrombus. Several endogenous antiplasmins are known. The drugs are used to control massive hemorrhage and in other coagulation disorders.Fibrinolysin: A product of the lysis of plasminogen (profibrinolysin) by PLASMINOGEN activators. It is composed of two polypeptide chains, light (B) and heavy (A), with a molecular weight of 75,000. It is the major proteolytic enzyme involved in blood clot retraction or the lysis of fibrin and quickly inactivated by antiplasmins.Thrombophlebitis: Inflammation of a vein associated with a blood clot (THROMBUS).Point Mutation: A mutation caused by the substitution of one nucleotide for another. This results in the DNA molecule having a change in a single base pair.Chromogenic Compounds: Colorless, endogenous or exogenous pigment precursors that may be transformed by biological mechanisms into colored compounds; used in biochemical assays and in diagnosis as indicators, especially in the form of enzyme substrates. Synonym: chromogens (not to be confused with pigment-synthesizing bacteria also called chromogens).Thromboembolism: Obstruction of a blood vessel (embolism) by a blood clot (THROMBUS) in the blood stream.Structure-Activity Relationship: The relationship between the chemical structure of a compound and its biological or pharmacological activity. Compounds are often classed together because they have structural characteristics in common including shape, size, stereochemical arrangement, and distribution of functional groups.Horseshoe Crabs: An arthropod subclass (Xiphosura) comprising the North American (Limulus) and Asiatic (Tachypleus) genera of horseshoe crabs.Plasminogen: Precursor of plasmin (FIBRINOLYSIN). It is a single-chain beta-globulin of molecular weight 80-90,000 found mostly in association with fibrinogen in plasma; plasminogen activators change it to fibrinolysin. It is used in wound debriding and has been investigated as a thrombolytic agent.Swine: Any of various animals that constitute the family Suidae and comprise stout-bodied, short-legged omnivorous mammals with thick skin, usually covered with coarse bristles, a rather long mobile snout, and small tail. Included are the genera Babyrousa, Phacochoerus (wart hogs), and Sus, the latter containing the domestic pig (see SUS SCROFA).Hemodilution: Reduction of blood viscosity usually by the addition of cell free solutions. Used clinically (1) in states of impaired microcirculation, (2) for replacement of intraoperative blood loss without homologous blood transfusion, and (3) in cardiopulmonary bypass and hypothermia.Calcium Chloride: A salt used to replenish calcium levels, as an acid-producing diuretic, and as an antidote for magnesium poisoning.Viperidae: A family of snakes comprising three subfamilies: Azemiopinae (the mountain viper, the sole member of this subfamily), Viperinae (true vipers), and Crotalinae (pit vipers). They are widespread throughout the world, being found in the United States, Central and South America, Europe, Asia and Africa. Their venoms act on the blood (hemotoxic) as compared to the venom of elapids which act on the nervous system (neurotoxic). (Goin, Goin, and Zug, Introduction to Herpetology, 3d ed, pp333-36)Rabbits: The species Oryctolagus cuniculus, in the family Leporidae, order LAGOMORPHA. Rabbits are born in burrows, furless, and with eyes and ears closed. In contrast with HARES, rabbits have 22 chromosome pairs.Lymphangiosarcoma: A malignant tumor originating from the endothelial cells of lymphatic vessels. Most lymphangiosarcomas arise in an arm secondary to radical mastectomy but they sometimes complicate idiopathic lymphedema. The lymphedema has usually been present for 6 to 10 years before malignant changes develop. (From Dorland, 27th ed; Holland et al., Cancer Medicine, 3d ed, p1866)Laser Coagulation: The use of green light-producing LASERS to stop bleeding. The green light is selectively absorbed by HEMOGLOBIN, thus triggering BLOOD COAGULATION.Glycoproteins: Conjugated protein-carbohydrate compounds including mucins, mucoid, and amyloid glycoproteins.Russell's Viper: A genus of snakes of the family VIPERIDAE. It is distributed in West Pakistan, most of India, Burma, Ceylon, Thailand, southeast China, Taiwan, and a few islands of Indonesia. It hisses loudly when disturbed and strikes with great force and speed. Very prolific, it gives birth to 20-60 young. This viper is the leading cause of snakebite in India and Burma. (Moore: Poisonous Snakes of the World, 1980, p127)Arthropod Proteins: Proteins synthesized by organisms belonging to the phylum ARTHROPODA. Included in this heading are proteins from the subdivisions ARACHNIDA; CRUSTACEA; and HORSESHOE CRABS. Note that a separate heading for INSECT PROTEINS is listed under this heading.Peptide Hydrolases: Hydrolases that specifically cleave the peptide bonds found in PROTEINS and PEPTIDES. Examples of sub-subclasses for this group include EXOPEPTIDASES and ENDOPEPTIDASES.Invertebrate Hormones: Hormones produced by invertebrates, usually insects, mollusks, annelids, and helminths.Polygeline: A 3.5 per cent colloidal solution containing urea-cross-linked polymerized peptides. It has a molecular weight of approximately 35,000 and is prepared from gelatin and electrolytes. The polymeric solution is used as a plasma expander.Endothelium, Vascular: Single pavement layer of cells which line the luminal surface of the entire vascular system and regulate the transport of macromolecules and blood components.Electrocoagulation: Procedures using an electrically heated wire or scalpel to treat hemorrhage (e.g., bleeding ulcers) and to ablate tumors, mucosal lesions, and refractory arrhythmias. It is different from ELECTROSURGERY which is used more for cutting tissue than destroying and in which the patient is part of the electric circuit.Enzyme-Linked Immunosorbent Assay: An immunoassay utilizing an antibody labeled with an enzyme marker such as horseradish peroxidase. While either the enzyme or the antibody is bound to an immunosorbent substrate, they both retain their biologic activity; the change in enzyme activity as a result of the enzyme-antibody-antigen reaction is proportional to the concentration of the antigen and can be measured spectrophotometrically or with the naked eye. Many variations of the method have been developed.Arginine: An essential amino acid that is physiologically active in the L-form.Chromatography, Gel: Chromatography on non-ionic gels without regard to the mechanism of solute discrimination.CHO Cells: CELL LINE derived from the ovary of the Chinese hamster, Cricetulus griseus (CRICETULUS). The species is a favorite for cytogenetic studies because of its small chromosome number. The cell line has provided model systems for the study of genetic alterations in cultured mammalian cells.Dogs: The domestic dog, Canis familiaris, comprising about 400 breeds, of the carnivore family CANIDAE. They are worldwide in distribution and live in association with people. (Walker's Mammals of the World, 5th ed, p1065)Viper Venoms: Venoms from SNAKES of the viperid family. They tend to be less toxic than elapid or hydrophid venoms and act mainly on the vascular system, interfering with coagulation and capillary membrane integrity and are highly cytotoxic. They contain large amounts of several enzymes, other factors, and some toxins.Genetic Therapy: Techniques and strategies which include the use of coding sequences and other conventional or radical means to transform or modify cells for the purpose of treating or reversing disease conditions.Cells, Cultured: Cells propagated in vitro in special media conducive to their growth. Cultured cells are used to study developmental, morphologic, metabolic, physiologic, and genetic processes, among others.Blood Loss, Surgical: Loss of blood during a surgical procedure.Drug Contamination: The presence of organisms, or any foreign material that makes a drug preparation impure.Fibrinolytic Agents: Fibrinolysin or agents that convert plasminogen to FIBRINOLYSIN.Mutagenesis, Site-Directed: Genetically engineered MUTAGENESIS at a specific site in the DNA molecule that introduces a base substitution, or an insertion or deletion.Disease Models, Animal: Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.Vitamin K Epoxide Reductases: OXIDOREDUCTASES which mediate vitamin K metabolism by converting inactive vitamin K 2,3-epoxide to active vitamin K.Plasma Kallikrein: A peptidohydrolytic enzyme that is formed from PREKALLIKREIN by FACTOR XIIA. It activates FACTOR XII; FACTOR VII; and PLASMINOGEN. It is selective for both ARGININE and to a lesser extent LYSINE bonds. EC 3.4.21.34.Snakes: Limbless REPTILES of the suborder Serpentes.
... is major protein domain of many blood coagulation factors. Blood coagulation factors V and VIII contain a C-terminal, twice ... Davie EW, Kane WH (1986). "Cloning of a cDNA coding for human factor V, a blood coagulation factor homologous to factor VIII ... "Synthetic factor VIII peptides with amino acid sequences contained within the C2 domain of factor VIII inhibit factor VIII ... Davie EW, Kane WH (1988). "Blood coagulation factors V and VIII: structural and functional similarities and their relationship ...
In the blood coagulation pathway, thrombin acts to convert factor XI to XIa, VIII to VIIIa, V to Va, fibrinogen to fibrin, and ... Warfarin and related drugs inhibit vitamin K-dependent carboxylation of several coagulation factors, including prothrombin. ... activated blood-coagulation factor II, blood-coagulation factor IIa, factor IIa, E thrombin, beta-thrombin, gamma-thrombin) is ... "The life cycle of coagulation factor VIII in view of its structure and function". Blood. 92 (11): 3983-96. PMID 9834200. Plow ...
... of Tyr1680 of human blood coagulation factor VIII is essential for the interaction of factor VIII with von Willebrand factor". ... coagulation factors, serine protease inhibitors, extracellular matrix proteins, and hormones. Tyrosine O-sulfate is a stable ... It has been shown that the sulfation of Tyr1680 in Factor VIII is essential for effective binding to vWF. Thus, when this is ...
"Interaction of coagulation factor VIII with members of the low-density lipoprotein receptor family follows common mechanism and ... This binding of THBS1, however, does not induce the subsequent degradation of these transcription factors, as reelin does, and ... Blood Coagul. Fibrinolysis. 19 (2): 166-77. doi:10.1097/MBC.0b013e3282f5457b. PMID 18277139. Ananyeva NM, Makogonenko YM, ... low density lipoprotein receptor and low-density lipoprotein receptor-related protein are shared within coagulation factor VIII ...
... factor VII comes into contact with tissue factors which starts a process called the blood coagulation cascade. Its purpose is ... Furthermore, it activates factors V, VIII and XI, all by cleaving the sequences GlyGlyGlyValArg-GlyPro and PhePheSerAlaArg- ... Most of those drugs are in the class of direct factor Xa inhibitors, but there is one DTI called AZD0837, which is a follow-up ... Thrombin also activates factor XIII that stabilizes the fibrin complex and therefore the clot and it stimulates platelets, ...
"Activation of human blood coagulation factor XI independent of factor XII. Factor XI is activated by thrombin and factor XIa in ... Like many other coagulation factors, it is a serine protease. In humans, Factor XI is encoded by the F11 gene. Factor XI (FXI) ... "Coagulation factor XI: a database of mutations and polymorphisms associated with factor XI deficiency". Blood Coagulation & ... "Expression of human blood coagulation factor XI: characterization of the defect in factor XI type III deficiency". Blood. 79 (6 ...
... participates in blood coagulation; it is a cofactor for factor IXa which, in the presence of Ca2+ and phospholipids ... the incidence of these inhibitors is dependent of various factors, including the factor VIII product itself. In the 1980s, some ... Factor VIII (FVIII) is an essential blood-clotting protein, also known as anti-hemophilic factor (AHF). In humans, factor VIII ... "NIH: F8 - coagulation factor VIII". National Institutes of Health. "Entrez Gene: F8 coagulation factor VIII, procoagulant ...
"Hepatocytes express blood coagulation factor XII (Hageman factor)". The Journal of Laboratory and Clinical Medicine. 115 (4): ... All of this, including the mechanism of inheritance, also holds true for the other contact factors, prekallikrein (Fletcher ... Coagulation factor XII, also known as Hageman factor, is a plasma protein. It is the zymogen form of factor XIIa, an enzyme (EC ... Factor XII is part of the coagulation cascade and activates factor XI and prekallikrein in vitro. Factor XII itself is ...
Factors VII, IX, and X all play key roles in blood coagulation and also share a common domain architecture. The factor IX ... and a Factor VIII cofactor, it hydrolyses one arginine-isoleucine bond in factor X to form factor Xa. Factor IX is inhibited by ... "Home: BeneFIX Coagulation Factor IX (Recombinant) Official Site". EMA: Idelvion "Home: Alprolix [Coagulation Factor IX ( ... "The life cycle of coagulation factor VIII in view of its structure and function". Blood. 92 (11): 3983-96. PMID 9834200. Lowe ...
The best known function of tissue factor is its role in blood coagulation. The complex of TF with factor VIIa catalyzes the ... pathway which involves both activated factor IX and factor VIII. Both pathways lead to the activation of factor X (the common ... the interface between factor Xa and tissue factor in the quaternary complex tissue factor-factor VIIa-factor Xa-tissue factor ... Engelmann B (2007). "Initiation of coagulation by tissue factor carriers in blood". Blood Cells, Molecules & Diseases. 36 (2): ...
doi:10.1182/blood-2002-01-0290. PMID 12393635. Duga S, Asselta R, Tenchini ML (August 2004). "Coagulation factor V". The ... Until the discovery of factor V, coagulation was regarded as a product of four factors: calcium (IV) and thrombokinase (III) ... and is homologous to coagulation factor VIII. The gene spans 70 kb, consists of 25 exons, and the resulting protein has a ... Factor V (pronounced factor five) is a protein of the coagulation system, rarely referred to as proaccelerin or labile factor. ...
Dutta-Roy, A.; Ray, T.; Sinha, A. (1986). "Prostacyclin stimulation of the activation of blood coagulation factor X by ... growth factor secretion and expression of several adhesive factors. In addition, hyperactive platelets are involved in the well ... Impact of Maternal Nutrition Early Nutrition and Lifestyle Factors: Effects on First Trimester Placenta Human Blood Platelet ... Dutta-Roy, A. K.; Sinha, A. K. (1987). "Purification and properties of prostaglandin E1/prostacyclin receptor of human blood ...
Chapter 38 Coagulation Factors V and VIII by GC White and GE Gilbert Archived 2017-09-11 at the Wayback Machine. in Blood: ... Factor VIII is used in haemophilia A and factor IX in haemophilia B. Factor replacement can be either isolated from human blood ... such as porcine factor VIII.[citation needed] If a person becomes refractory to replacement coagulation factor as a result of ... the Y-chromosome in the male has no gene for factors VIII or IX. If the genes responsible for production of factor VIII or ...
In 1959 she was working on the preparation of human coagulation factors at the Medical Research Council Blood Coagulation ... Two years later, she began to study plasma concentration and selective extraction of factor VIII. By 1953 she had devised a ... "Oxford, the Mecca for blood coagulation research in the 1950s and 1960s". Thromb Haemost. 2: 2085-2088. 2004. Tilli Tansey; ... Dr Ethel Bidwell (1919-2003) was a British research scientist who investigated blood coagulation. In 1950, Bidwell, an enzyme ...
TAP and antistasin were used to estimate factor Xa as a drug target. Blood coagulation is a complex process by which the blood ... Factors IIa, Xa, VIIa, IXa and XIa are all proteolytic enzymes that have a specific role in the coagulation cascade. Factor Xa ... Heparin targets multiple factors in the blood coagulation cascade, one of them being FXa. At first, it had many side effects ... Factor Xa is an activated serine protease that occupies a key role in the blood coagulation pathway by converting prothrombin ...
... bypasses factors VIII and IX and causes coagulation without the need for factors VIII and IX. It is important for some patients ... Recombinant factor VIIa (rFVIIa) is a form of blood factor VII that has been manufactured via recombinant technology. The most ... In people with hemophilia type A and B who have a deficiency of factors VIII and IX, these two factors are administered for ... These NAbs increase over time and inhibit the action of coagulation in the body. rFVIIa, which is an activated form of factor ...
Deficiencies of coagulation factors are associated with clinical bleeding. For instance, deficiency of Factor VIII causes ... There are several named coagulation factors that interact in a complex way to form blood clots, as discussed in the article on ... Deficiencies in other factors, such as factor XIII or factor VII are occasionally seen, but may not be associated with severe ... Antibodies to Factor VIII can also inactivate the Factor VII and precipitate bleeding that is very difficult to control. This ...
Tissue factor also binds to factor VII in the blood, which initiates the intrinsic coagulation cascade to increase thrombin ... along with the coagulation factors) is to react to bleeding from blood vessel injury by clumping, thereby initiating a blood ... Alpha granules contain clotting mediators such as factor V, factor VIII, fibrinogen, fibronectin, platelet-derived growth ... Platelet-coagulation factor interactions: coagulation facilitation[edit]. Platelet activation causes its membrane surface to ...
Some of the proteins synthesized by the liver include coagulation factors I (fibrinogen), II (prothrombin), V, VII, VIII, IX, X ... Examples of highly liver-specific proteins include apolipoprotein A II, coagulation factors F2 and F9, complement factor ... The liver plays a role in the production of clotting factors as well as red blood cell production. ... The hepatic artery carries oxygen-rich blood from the aorta, whereas the portal vein carries blood rich in digested nutrients ...
... stasis of blood, vessel wall injury, and altered blood coagulation.[9][10] Some risk factors predispose for venous thrombosis ... The main mechanism is exposure of tissue factor to the blood coagulation system.[22] Inflammatory and other stimuli (such as ... altered coagulation. [14] Hyperhomocysteinemia. altered coagulation. [14] Elevated factors II, VIII, IX, XI. altered ... Disturbed blood flow[edit]. Further information: Blood flow. Causes of disturbed blood flow include stagnation of blood flow ...
Blood coagulation factors[edit]. *Coagulation factor VIIIα. *Coagulation factor IXα ... Blood products of human origin and plasma substitutes[edit]. Blood and blood components[edit]. ... 11 Blood products of human origin and plasma substitutes *11.1 Blood and blood components ... Anti-vascular endothelial growth factor (VEGF)[edit]. *Bevacizumabα. Medicines for reproductive health and perinatal care[edit] ...
... bypasses factors VIII and IX and causes coagulation without the need for factors VIII and IX. It can't be given without ... Recombinant factor VIIa (rFVIIa) is a form of blood factor VII that has been manufactured via recombinant technology. The most ... In people with hemophilia type A and B who have a deficiency of factors VIII and IX, these two factors are administered for ... This treatment results in activation of the extrinsic pathway of blood coagulation. ...
All factors in the coagulation cascade. While the endothelium does produce some factor VIII, the majority of factor VIII is ... The liver plays the major role in producing proteins that are secreted into the blood, including major plasma proteins, factors ... Inhibitors of coagulation:, inactivate an enormous variety of proteinases α2-macroglobulin α1-antitrypsin Antithrombin III ... Angiotensinogen, when converted to angiotensin causes vasoconstriction and release of aldosterone, in effect increasing blood ...
Whole blood coagulation thrombelastographic profiles employing minimal tissue factor activation. J Thromb Haemost. 2003;1:551-8 ... Prolongation of CT may be a result of coagulation deficiencies, primarily coagulation factors, or heparin (dependent on the ... experience of eight years and 829 LTX. Joint International Congress of ILTS, ELITA & LICAGE, July 9-12, 2008, Paris, France ( ... Whole blood thrombelastographic coagulation profiles using minimal tissue factor activation can display hypercoagulation in ...
The motifs coagulation factor 5/8 type C or discoidin and thrombospondin type 1 repeat (TSR) present in SCO-spondin consensus ... it is known to be linked to the different domains in SCO-spondin that are related to coagulation factors and TSRs, as referred ... characterized by numerous blood capillaries and glial cells, projects into the local blood vessels and to the subarachnoidal ... a coagulation factor 5/8 type C (FA5-8C) or discoidin motif and a C-terminal cystin knot (CTCK) domain provides a wider insight ...
... and abnormalities in blood clotting often consistent with disseminated intravascular coagulation (DIC) such as a prolonged ... proteins including interferon regulatory factor 3 and interferon regulatory factor 7 trigger a signalling cascade that leads to ... have also been tried including heparin in an effort to prevent disseminated intravascular coagulation and clotting factors to ... Four people died, and four people survived; five of these eight cases were laboratory-confirmed. A total of 583 contacts were ...
Decreased Factor VII Coagulation Activity. Normal Factors VIII, IX, X, XI, XII.. ... The appropriate ratio of anticoagulant to blood in the testing vessel is also essential. Additionally, the correct ... These values include a decreased factor VII coagulant activity (FVII:C) and normal coagulant activities for factors II, IX, and ... Acquired factor VII deficiency must be differentiated from inherited factor VII deficiency and from acquired disorders due to ...
Genotypes of patients with factor VII deficiency Mutation (Name) -96 C>T IVS4+1 G>A* Location Domain Type Genotype Origin ... Blood 84:2214, Toso R, Pinotti M, High KA, et al: A frequent human coagulation factor VII mutation (A294V, c152) in loop 140s ... Topologically equivalent mutations causing dysfunctional coagulation factors VII ( 294 Ala Val) and X ( 334 Ser Pro). Hum Mol ... Blood Coagul Fibrinolysis 21:775, 2010.. 21 79. James HL, Girolami A, Hubbard JG, et al: The dysfunction of coagulation factor ...
However, it had no effect on T. tridentatus clotting enzyme and factor C, mammalian blood coagulation factors (activated ... However, it had no effect on T. tridentatus clotting enzyme and factor C, mammalian blood coagulation factors (activated ... However, it had no effect on T. tridentatus clotting enzyme and factor C, mammalian blood coagulation factors (activated ... However, it had no effect on T. tridentatus clotting enzyme and factor C, mammalian blood coagulation factors (activated ...
Von Willebrand factor (vWF) is stored in endothelial cells and released into blood plasma upon vascular dysfunction. This meta- ... Plasma vWF level in CAD patients examined at 24 h and 48 h after admission might be an independent prognostic factor for MACE. ... vWF adheres platelets to the blood vessel wall, and acts as a plasma carrier for factor VIII to prevent its degradation in the ... Eikelboom J. Clopidogrel does not suppress blood markers of coagulation activation in aspirin-treated patients with non-ST- ...
Hemorrhagic disorder due to an Isoniazid-associated acquired factor XIII inhibitor in a patient with Waldenstroms ... a risk factor for hemorrhagic and non-hemorrhagic stroke. ... the fibrin stabilizing factor that helps in blood coagulation. ... Risk factors for postoperative infection. *Abstracts: Cytotoxic suppression of acquired factor VIII:C inhibitors. Acquired ... Coagulation studies revealed a deficiency in factor XIII activity, and further tests indicated the presence of a factor XIII ...
Predicting mortality using nonbiological factors. A study identifies behavioral, psychological, and social factors that could ... is caused by various mutations in the blood coagulation factor VIII (F8) gene. Among the genotypes that result in hemophilia A ... is often caused by chromosomal inversions that involve a portion of the blood coagulation factor VIII (F8) gene that encodes ... Targeted inversion and reversion of the blood coagulation factor 8 gene in human iPS cells using TALENs. Chul-Yong Park, ...
... initiates blood coagulation by promoting FXa generation (extrinsic-Xa). Subsequent generation of intrinsic FXa … ... CrossRefPubMed Komiyama Y, Pedersen AH, Kisiel W. Proteolytic activation of human factors IX and X by recombinant human factor ... Proteolysis of blood coagulation factor VIII by the factor VIIa-tissue factor complex: generation of an inactive factor VIII ... Proteolysis of blood coagulation factor VIII by the factor VIIa-tissue factor complex: generation of an inactive factor VIII ...
ABO blood group was determined by indirect technique. FVIII:C and Fbg were measured by clotting assays. VWF antigen (VWF:Ag), ... Conclusions ABO blood group, age, and gender showed different effects on plasma levels of FVIII:C, Fbg, VWF:Ag, VWF:CBA, VWF: ... and von Willebrand factor (VWF). Age and gender have been shown to influence FVIII, VWF, fibrinogen (Fbg), and ADAMTS13 (A ... Background ABO blood group is a hereditary factor of plasma levels of coagulation factor VIII (FVIII) ...
The inability for vWF to bind fVIII is the basis for another blood coagulation disorder, von Willebrands disease type 2N, ... Rather, changes in B-factor ratios between the C2 complexes and the isolated C2 domain suggest that dynamic changes upon 3E6 ... which can lead to hemophilia-type levels of fVIII in the blood and subsequent bleeding episodes. Previous publications have ... A deficiency in blood coagulation factor VIII (fVIII) is responsible for the inherited bleeding disorder hemophilia A, which ...
Factor VIII participates in blood coagulation; it is a cofactor for factor IXa which, in the presence of Ca2+ and phospholipids ... the incidence of these inhibitors is dependent of various factors, including the factor VIII product itself. In the 1980s, some ... Factor VIII (FVIII) is an essential blood-clotting protein, also known as anti-hemophilic factor (AHF). In humans, factor VIII ... "NIH: F8 - coagulation factor VIII". National Institutes of Health. "Entrez Gene: F8 coagulation factor VIII, procoagulant ...
Sulfation of Tyr1680 of human blood coagulation factor VIII is essential for the interaction of factor VIII with von Willebrand ... Regions 301-303 and 333-339 in the catalytic domain of blood coagulation factor IX are factor VIII-interactive sites involved ... Identification of a binding site for blood coagulation factor IXa on the light chain of human factor VIII ... a bispecific antibody recognizing coagulation factors IX and X: how does it actually compare to factor VIII?. Blood 2017; 130 ( ...
Coagulation factors are a group of related proteins that are essential for the formation of blood clots. Learn about this gene ... The F8 gene provides instructions for making a protein called coagulation factor VIII. ... The F8 gene provides instructions for making a protein called coagulation factor VIII. Coagulation factors are a group of ... The active protein (sometimes written as coagulation factor VIIIa) interacts with another coagulation factor called factor IX. ...
Blood-coagulation factor VIII. Antihemophilic factor that is part of the factor VIII/von Willebrand factor complex. Factor VIII ... is produced in the liver and acts in the intrinsic pathway of blood coagulation. It serves as a cofactor in factor X activation ... Blood Coagulation Factors: 2147*Factor VIII: 5450*human F8 protein: 163. *von Willebrand factor drug combination factor VIII: ... Blood-coagulation factor VIII. Antihemophilic factor that is part of the factor VIII/von Willebrand factor complex. Factor VIII ...
Octanate belongs to a group of medicines called clotting factors and contains human blood. coagulation factor VIII. This is a ... Human Coagulation Factor VIII. Octanate 100 IU/ml Powder and Solvent for Solution for Injection. Human Coagulation Factor VIII ... dosage of factor VIII is expressed in IU.. One IU of factor VIII activity is equivalent to the amount of factor VIII in one ml ... maintain factor VIII activity at 30%. to 60%.. Your response to factor VIII products may vary. The factor VIII level in your ...
Enhanced uptake of blood coagulation factor VIII containing immune complexes by antigen presenting cells. ... Risk factors for frequent severe exacerbations in late-onset eosinophilic asthma.. de Groot JC, Amelink M, de Nijs SB, Plaat R ... Limited promiscuity of HLA-DRB1 presented peptides derived of blood coagulation factor VIII. ... von Willebrand factor binds to the surface of dendritic cells and modulates peptide presentation of factor VIII. ...
The method prevents complex formation between tissue factor and factor VII/VIIa and thus inhibits coagulation of blood in ... Anti-tissue factor monoclonal antibodies produced by hybridoma cell lines TFS-5G9 or TF9-6B4 may be used in the claimed methods ... of a therapeutically effective amount of a monoclonal antibody which inhibits the ability of tissue factor to bind to factor ... This invention provides a method of inhibiting coagulation in extracorporeal circulation in a subject, comprising ...
Deficiency of blood coagulation Factors VIII, IX, or XI is associated with hemophilia. Factor XII (FXII) autoactivates by ... Using patient blood samples and isolated proteins, we identified a novel class of Factor XII activators. Factor XII was ... Consistent with this, we found that Factor XII, but not Factor XI, was activated and kallikrein was formed in blood from ... This cascade is initiated by autoactivation of Factor XII and leads to both coagulation (via Factor XI) and an inflammatory ...
The C1 and C2 domains of blood coagulation factor VIII mediate its endocytosis by dendritic cells. ... Predisposing factors, pathogenesis and therapeutic intervention of Kawasaki disease.. Galeotti C, Kaveri SV, Cimaz R, Koné-Paut ... Role of the factor VIII-binding capacity of endogenous von Willebrand factor on the development of factor VIII inhibitors in ... Prevention of the anti-factor VIII memory B-cell response by inhibition of the Brutons tyrosine kinase in experimental ...
Agent neutralizint tissue factor inhibitor and agent neutralizing activated blood coagulation factor viii preparation. October ... The selected dosage level will depend upon a variety of factors including the activity of the particular extract of the present ... Tumor necrosis factor-α (TNF-α), a pleiotropic cytokine, is produced mainly by macrophages, but it may be produced by other ... Tumor necrosis factor-α (TNF-α) has been implicated as a mediator in inflammatory bowel disease, rheumatoid arthritis, juvenile ...
... which is a protein that is essential for the process of blood clotting (coagulation). ... Helixate Nexgen contains the active ingredient factor VIII, ... coagulation).. Factor VIII is found in human blood plasma and ... Lack of any one of these clotting factors can lead to uncontrolled bleeding or excessive bruising following injury. ... Helixate Nexgen (factor VIII). Helixate Nexgen contains the active ingredient factor VIII, which is a protein that is essential ...
coagulation factor VIII, which is necessary for a normal course of blood. coagulation. After reconstitution of the powder with ... If you have existing cardiovascular risk factors, therapy with Haemoctin may. increase the cardiovascular risk. If you are ... assay for determining factor VIII activity in patients blood samples, plasma. factor VIII activity results can be ... factor VIII in one ml of normal human plasma.. On demand treatment. The calculation of the required dose of factor VIII is ...
C07K14/745-Blood coagulation or fibrinolysis factors * C07K14/755-Factors VIII, e.g. factor VIII C (AHF), factor VIII Ag (VWF) ... non-degraded factor VIII/von-Willebrand factor during blood processing US4710381A (en) * 1984-05-22. 1987-12-01. The Blood ... Method for maintaining intact, non-degraded factor VIII/von-Willebrand factor during blood processing ... Activated factor VIII as a therapeutic agent and method of treating factor VIII deficiency ...
Factor VIII is one of the proteins involved in the blood coagulation (clotting) process. Patients with haemophilia A are more ... treatments to try and remove the inhibitors from the blood or medicines containing other coagulation factors such as factor ... Recombinant porcine factor VIII (B-domain-deleted) is a type of factor VIII that is expected to work in the same way as human ... This is because the pig factor VIII has a slightly different shape to human factor VIII and will not be as easily recognised by ...
ANTIBODY SUBSTITUTING FOR FUNCTION OF BLOOD COAGULATION FACTOR VIII. The present inventors produced a variety of bispecific ... comprising the application of certain blood coagulation (clotting) factors in... ... Combination Therapy with Coagulation Factors and Multispecific Antibodies. The invention relates to therapies for a patient ...
... recessive disorder caused by deficiency of functional plasma clotting factor VIII (FVIII). In a significant number of cases, ... Laboratory studies for suspected hemophilia include a complete blood cell count, coagulation studies, and a factor VIII (FVIII ... Hemophilia A and parahemophilia: deficiencies of coagulation factors VIII and V. Scriver CR, Beaudet AL, Sly WS, Valle D, eds. ... 3-Dimensional structure of membrane-bound coagulation factor VIII: modeling of the factor VIII heterodimer within a 3- ...
  • This protease inhibitor inhibited stoichiometrically the amidase activity of trypsin (K 1 =4.60×10 -10 M), and also had inhibitory effects on α-chymotrypsin (K 1 =5.54×10 -9 M), elastase (K 1 =7.20×10 -8 M), plasmin, and plasma kallikrein. (elsevier.com)
  • Anti-tissue factor monoclonal antibodies produced by hybridoma cell lines TFS-5G9 or TF9-6B4 may be used in the claimed methods. (google.com)
  • If you start the treatment with Haemoctin, it is possible that your immune system develops antibodies (inhibitors) to factor VIII. (drugs.com)
  • However, some patients with haemophilia A could not benefit from these medicines because their immune system (the body's natural defences) had reacted by producing 'inhibitors' (antibodies) against factor VIII. (europa.eu)
  • Blood cells are suspended in the plasma, which is made up of water and dissolved materials, including hormones, antibodies, and enzymes that are being carried to the tissues, and cellular waste products that are being carried to the lungs and kidneys. (labcorp.com)
  • Analyses of blood samples for the presence or absence of antibodies or antigens are used in the diagnosis of diseases, such as HIV infection, hepatitis, Lyme disease, prenatal profiles including TORCH (an acronym for: "Toxoplasmosis, Rubella, Cytomegalovirus, Herpes") profiles, as well as other infectious disease profiles. (google.com.au)
  • Miesbach W, Voigt J, Peetz D, Scharrer I (2003) Massive bleeding symptoms in two patients with factor V inhibitor and antiphospholipid antibodies after treatment with ciprofloxacin. (springer.com)
  • Examples hereof are concizumab, a monoclonal antibody targeting tissue factor pathway inhibitor, and fitusiran, an small interfering RNA approach to reduce expression of antithrombin. (ashpublications.org)
  • Effective treatment of bleeding symptoms should be based upon the clinical situation and depends on the inhibitor characteristics against human and porcine factor VIII. (curehunter.com)
  • Immunosuppression usually does not significantly affect the disappearance of the factor VIII inhibitor antibody. (curehunter.com)
  • Cases of recurrent inhibitors have been observed after switching from one factor VIII product to another in previously treated patients with more than 100 exposure days who have a previous history of inhibitor development. (drugs.com)
  • Bayani N, Rugina M, Haddad-Vergnes L, Lelong F (2002) High-titer acquired factor V inhibitor responsive to corticosteroids and cyclophosphamide in a patient with two malignant tumors. (springer.com)
  • To examine the in vitro regulation of hepatocyte growth factor activator inhibitor type 2 (HAI-2) in breast cancer cells and the in vivo predictive role for the efficacy of chemoendocrine primary therapy in patients with breast cancer. (aacrjournals.org)
  • Although investigating the role of hypoxic regulation of HGF via hypoxia-inducible factor 1α, we hypothesized that there may be reciprocal regulation of its inhibitor, HAI-2. (aacrjournals.org)
  • ABSTRACT Factor VII gene polymorphisms may contribute to elevations in factor VII coagulant (FVIIc) levels that have been associated with cardiovascular risk. (who.int)
  • Each vial of ADYNOVATE is labeled with the actual factor VIII activity in IU determined using one-stage clotting assay, using a reference material calibrated against a World Health Organization (WHO) International Standard for factor VIII concentrates. (rxlist.com)
  • Plasma factor VIII levels can be monitored clinically using a one-stage clotting assay. (rxlist.com)
  • A blood test for the assay of clotting factor VIII is frequently done to measure its activity inside the body. (brighthub.com)
  • Shanbhag S, Ghosh K, Shetty S. Genetic basis of severe factor XIII deficiency in a large cohort of Indian patients: Identification of fourteen novel mutations. (medscape.com)
  • Severe gastrointestinal vasculitis in Henoch-Schoenlein purpura: pathophysiologic mechanisms, the diagnostic value of factor XIII, and therapeutic options. (medscape.com)
  • In more severe cases, transfusions of blood platelets may be necessary. (rarediseases.org)
  • the inclusion of steps in the processing of the blood or plasma that can inactivate or remove viruses. (drugs.com)
  • Both the model and parameters to which model sensitivity is being analyzed provide newer insights into blood coagulation: the model incorporates distinct equations for plasma-phase and platelet membrane-bound species, and sensitivity to initial concentrations is a new dimension in sensitivity analysis. (springer.com)
  • In the average adult male there are approximately 5 quarts (4.75 liters) of blood, composed of about 3 quarts (2.85 liters) of plasma and 2 quarts (1.9 liters) of cells. (labcorp.com)
  • Either plasma or serum may be separated from the blood cells by centrifugation. (labcorp.com)
  • Plasma is obtained from blood that has been mixed with an anticoagulant in the collection tube and has, therefore, not clotted. (labcorp.com)
  • Duckert F. Documentation of the plasma factor XIII deficiency in man. (medscape.com)
  • Solvent/detergent-treated pooled plasma is ABO blood type specific and offers more protection to patients than is found in standard FFP. (medscape.com)
  • By 1937, however, it was found that substances dissolved in blood plasma, the liquid part of blood, were a necessary part of the normal clotting process. (encyclopedia.com)
  • It was not until 1965, however, that another doctor discovered a way to separate the protein factors from the liquid part of blood plasma by a freeze-drying process. (encyclopedia.com)
  • An eluate from Al(OH)(3), which has been incubated with normal plasma, supplies factors X and IX in their ;plasma' (unactivated) form with II. (tripdatabase.com)
  • Calculate the dose of ADYNOVATE based on the empirical finding that one international unit of ADYNOVATE per kg body weight increases the plasma factor VIII level by 2 IU per dL of plasma. (rxlist.com)
  • Periodic blood transfusions (or transfusions with plasma or other blood products that supply clotting factors), however, may be required following trauma or simple bleeding. (vetstreet.com)
  • 8 , 9 Alternative approaches are aiming to restore the hemostatic balance by interfering with anticoagulant pathways. (ashpublications.org)
  • The zebrafish is an excellent candidate for establishing genetic models of hyperlipidemia due to conservation of cell types and molecular pathways involved in lipid metabolism and cholesterol synthesis ( 8 , 14 - 20 ). (pubmedcentralcanada.ca)
  • To define the contribution of specific signal pathways to RGC development and optimize the differentiation of hPSCs toward RGCs, we examined RGC differentiation in three stages: (1) eye field progenitors expressing the eye field transcription factors (EFTFs), (2) RGC progenitors expressing MATH5, and (3) RGCs expressing BRN3B and ISLET1. (arvojournals.org)
  • Precise modulation of signaling pathways, including Wnt, insulin growth factor-1, and fibroblast growth factor, in combination with mechanical isolation of neural rosette cell clusters significantly enriched RX and PAX6 double-positive eye field progenitors from hPSCs by day 12. (arvojournals.org)
  • A recent systematic review of randomised controlled trials shows that TXA reduces the probability of receiving a blood transfusion by nearly 40% [ 1 ]. (biomedcentral.com)