Article
Factor XIII Deficiency.
Seminars in thrombosis and hemostasis - 1 Jun 2009
Karimi Mehran, Bereczky Zsuzsanna, Cohan Nader, Muszbek László
Abstract excerpt
Factor XIII (FXIII) is a tetrameric zymogen (FXIII-A (2)B (2)) that is converted into an active transglutaminase (FXIIIa) by thrombin and Ca (2+) in the terminal phase of the clotting cascade. By cross-linking fibrin chains and alpha (2) plasmin inhibitor to fibrin, FXIIIa mechanically stabilizes fibrin and protects it from fibrinolysis. Severe deficiency of the potentially active A subunit (FXIII-A) is a rare...
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