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Encyclopedia > Antithrombin

Image:Antithrombin.png|right|framed|Antithrombin drawn from PDB 1E03.]] The Protein Data Bank (PDB) is a repository for 3-D structural data of proteins and nucleic acids. ...


Antithrombin is a small molecule that inactivates several enzymes of the coagulation system. It is a glycoprotein produced by the liver. The coagulation of blood is a complex process during which blood forms solid clots. ... A glycoprotein is a macromolecule composed of a protein and a carbohydrate (a sugar). ... The liver is one of the largest internal organs of the human body. ...

Contents


Function

Antithrombin is a serpin (serine protease inhibitor) that inactivates a number of enzymes from the coagulation system, namely the activated forms of Factor X, Factor IX, Factor II (thrombin), Factor VII, Factor XI, and Factor XII. Its affinity for these molecules (i.e. its effectivity) is enhanced by heparin. Serine protease inhibitors or serpins (short for serine protease inhibitor) are a group of proteins that inhibit peptidases (old name: proteases). ... Ribbon diagram of the enzyme TIM. TIM is catalytically perfect, meaning its conversion rate is limited, or nearly limited to its substrate diffusion rate. ... The coagulation of blood is a complex process during which blood forms solid clots. ... Factor X, also known by the eponym Stuart-Prower factor or as thrombokinase, is an enzyme ( EC 3. ... Factor IX (or Christmas factor or Christmas-Eve factor) is one of the serine proteases (EC 3. ... Thrombin (activated Factor II) is a coagulation protein that has many effects in the coagulation cascade. ... Heparin as a drug is used as an injectable anticoagulant. ...


Role in disease

Antithrombin deficiency is a rare hereditary disorder that generally comes to light when a patient suffers recurrent venous thrombosis and pulmonary embolism. This was first described by Egeberg in 1965. The patients are treated with anticoagulants or, more rarely, with antithrombin concentrate. A rare disease has such a low prevalence in a population that a doctor in a busy general practice would not expect to see more than one case a year. ... A genetic disorder, or genetic disease is a disease caused, at least in part, by the genes of the person with the disease. ... Thrombosis is the formation of a clot or thrombus inside a blood vessel, obstructing the flow of blood through the circulatory system. ... An anticoagulant is a substance that prevents coagulation; that is, it stops blood from clotting. ...


In renal failure, especially nephrotic syndrome, antithrombin is lost in the urine, leading to a higher activity of Factor II and Factor X and in increased tendency to thrombosis. Renal failure is the condition where the kidneys fail to function properly. ... Thrombosis is the formation of a clot or thrombus inside a blood vessel, obstructing the flow of blood through the circulatory system. ...


Genetics

The gene for antithrombin is located on the first chromosome, locus 1q23-q25.1. This stylistic schematic diagram shows a gene in relation to the double helix structure of DNA and to a chromosome (right). ... Figure 1: Chromosome. ...


Nomenclature

Antithrombin is officially called antithrombin III and is a member of a larger family of antithrombins (numbered I, II etc. to VI). All are serpins. Only AT III (and possibly AT I) is medically significant, with AT III generally referred to as antithrombin. Serine protease inhibitors or serpins (short for serine protease inhibitor) are a group of proteins that inhibit peptidases (old name: proteases). ...


Reference

  • Egeberg O. Inherited antithrombin deficiency causing thrombophilia. Thromb Diath Haemorrh 1965;13:516–520. PMID 14347873.

External links

  • Patient's pages on antithrombin
  • OMIM 107300 (antithrombin deficiency)


The Mendelian Inheritance in Man project is a database that catalogues all the known diseases with a genetic component, and - when possible - links them to the relevant genes in the human genome. ...

Coagulation
Coagulation factors: - Fibrin (I) - (Pro)thrombin (II) - FV - FVII - FVIII - FIX - FX - FXI - FXII - FXIII - HMWK - vWF - Tissue factor
Inhibitors: Antithrombin - Protein C - Protein S - Protein Z - ZPI - TFPI
Fibrinolysis: Plasmin - tPA/urokinase - PAI-1/2 - α2-AP - TAFI


[1] The coagulation of blood is a complex process during which blood forms solid clots. ... Fibrin is a protein involved in the clotting of blood. ... Thrombin (activated Factor II) is a coagulation protein that has many effects in the coagulation cascade. ... Factor V is a protein of the coagulation system, rarely referred to as proaccelerin or labile factor. ... Factor VII (old name proconvertin) is one of the central proteins in the coagulation cascade. ... Factor VIII (FVIII) is an essential clotting factor. ... Factor IX (or Christmas factor or Christmas-Eve factor) is one of the serine proteases (EC 3. ... Factor X, also known by the eponym Stuart-Prower factor or as thrombokinase, is an enzyme ( EC 3. ... Factor XI or plasma thromboplastin antecent is one of the enzymes ( EC 3. ... The Hageman factor, is a plasma protein now usually known as factor XII. It is part of the coagulation cascade and activates factor XI and prekallikrein. ... Factor XIII or fibrin stabilizing factor is an enzyme (EC 2. ... High-molecular weight kininogen (HMWK), also known as the Williams-Fitzgerald-Flaujeac factor or the Fitzgerald factor is a protein from the blood coagulation system as well as the kinin-kallikrein system. ... Von Willebrand factor is a blood glycoprotein of the coagulation system. ... Thromboplastin is a substance present in tissues, platelets, and leukocytes necessary for the coagulation of blood; in the presence of calcium ions thromboplastin is necessary for the conversion of prothrombin to thrombin, an important step in coagulation of blood. ... Protein C is a major physiological anticoagulant. ... Protein S is a vitamin K-dependent plasma glycoprotein synthesized in the liver and it functions as a cofactor to Protein C in the inactivation of Factors Va and VIIIa. ... Protein Z is a member of the coagulation cascade, the group of blood proteins that leads to the formation of blood clots. ... Protein Z-dependent protease inhibitor is a protein circulating in the blood which inhibits factors Xa and XIa of the coagulation cascade. ... Tissue Factor Pathway Inhibitor (or TFPI) is a protein that can reversibly inhibit Factor Xa (Xa), and after it has inhibted Xa, the Xa-TFPI complex can then inhibit the FVIIa-TF complex. ... Fibrinolysis is the process where a fibrin clot, the product of coagulation, is broken down. ... Plasmin is an important degrading enzyme (EC 3. ... In blood coagulation, tissue plasminogen activator (tPA) is an enzyme (EC 3. ... Urokinase, also called urokinase-type Plasminogen Activator (uPA) is an enzyme (EC 3. ... Plasminogen activator inhibitor-1 is the principal inhibitor of tissue plasminogen activator (tPA) and urokinase (uPA), the activators of plasminogen and hence fibrinolysis (the physiological breakdown of blood clots). ... Alpha 2-antiplasmin (or α2-antiplasmin or plasmin inhibitor) is a serine protease inhibitor (serpin) responsible for inactivating plasmin, an important enzyme that participates in fibrinolysis and degradation of various other proteins. ...


  Results from FactBites:
 
Antithrombin - Wikipedia, the free encyclopedia (279 words)
Antithrombin is a serpin (serine protease inhibitor) that inactivates a number of enzymes from the coagulation system, namely the activated forms of Factor X, Factor IX, Factor II (thrombin), Factor VII, Factor XI, and Factor XII.
Antithrombin deficiency is a rare hereditary disorder that generally comes to light when a patient suffers recurrent venous thrombosis and pulmonary embolism.
In renal failure, especially nephrotic syndrome, antithrombin is lost in the urine, leading to a higher activity of Factor II and Factor X and in increased tendency to thrombosis.
  More results at FactBites »

 
 

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