Article
Factor XIII deficiency: complete phenotypic characterization of two cases with novel causative mutations.
Haemophilia : the official journal of the World Federation of Hemophilia - 1 Jan 2014
Katona É, Muszbek L, Devreese K, Kovács K B, Bereczky Z, Jonkers M, Shemirani A H, Mondelaers V, Ermens A A M
Abstract excerpt
Coagulation factor XIII (FXIII) exists as heterotetramer (FXIII-A₂B₂) in the plasma and as dimer (FXIII-A₂) in cells. Activated FXIII mechanically stabilizes fibrin and protects it from fibrinolysis by cross-linking fibrin chains and α₂-plasmin inhibitor to fibrin. FXIII is essential to maintaining haemostasis, and its deficiency causes severe bleeding diathesis. Due to improper laboratory practices, FXIII...
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