Encyclopedia of Molecular Mechanisms of Disease

2009 Edition
| Editors: Florian Lang

Fibrinolytic Disorders

Reference work entry
DOI: https://doi.org/10.1007/978-3-540-29676-8_636
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Synonyms

Fibrinolytic defects; Abnormalities of the fibrinolytic system; Disfibrinolysis

Definition and Characteristics

Congenital or acquired abnormalities of the fibrinolytic system leading to hypo- or hyperfibrinolysis due to impaired activity of plasmin, its inhibitors, or plasminogen (PLG) activators.

Prevalence

Primary genetic disorders of fibrinolysis are uncommon, acquired fibrinolytic disorders are frequent, and secondary to other primary disorders or therapeutic interventions.

Genes

PLG coding for the plasminogen, localized or chromosome 6q26-6q27; SERPINF2 – alpha-2-plasmin inhibitor, 17p13; SERPINE1 – plasminogen activator inhibitor type 1, 7q21.3; PLAT – tissue plasminogen activator, 8p12-8p11; PLAU – urokinase-type plasminogen activator, 10q24; CPB2 – carboxypeptidase B2 (plasma) (thrombin-activatable fibrinolysis inhibitor), 13q14.1; UPAR – plasminogen activator receptor, urokinase-type (urokinase receptor), 19q13.2.

Molecular and Systemic Pathophysiology

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References

  1. 1.
    Cesarman-Maus G, Hajjar KA (2005) Molecular mechanisms of fibrinolysis. Br J Haematol 129:307–321PubMedGoogle Scholar
  2. 2.
    Schuster V, Hugle B, Tefs K (2007) Plasminogen defiency. J Thromb Haemost 5:2315–2322Google Scholar
  3. 3.
    Syrovets T, Simmet T (2004) Novel aspects and new roles for the serine protease plasmin. Cell Mol Life Sci 61:873–885PubMedGoogle Scholar
  4. 4.
    Longstaff C, Thelwell C (2005) Understanding the enzymology of fibrinolysis and improving thrombolytic therapy. FEBS Lett 579:3303–3309PubMedGoogle Scholar
  5. 5.
    Cale JM, Lawrence DA (2007) Structure-function relationships of plasminogen activator inhibitor-1 and its potential as a therapeutic agent. Curr Drug Targets 8:971–981PubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Berlin Heidelberg 2009

Authors and Affiliations

  1. 1.Department of BiotechnologyShemyakin-Ovchinnikov Institute of Bioorganic Chemistry RASMoscowRussia