Article
Altered fibrinolysis in autosomal dominant thrombomodulin-associated coagulopathy.
Blood - 6 Oct 2016
Burley Kate, Whyte Claire S, Westbury Sarah K, Walker Mary, Stirrups Kathleen E, Turro Ernest, Chapman Oliver G, Reilly-Stitt Christopher, Mutch Nicola J, Mumford Andrew D
Abstract excerpt
Thrombomodulin-associated coagulopathy (TM-AC) is a newly recognized dominant bleeding disorder in which a p.Cys537Stop variant in the thrombomodulin (TM) gene THBD, results in high plasma TM levels and protein C-mediated suppression of thrombin generation. Thrombin in complex with TM also activates thrombin-activatable fibrinolysis inhibitor (TAFI). However, the effect of the high plasma TM on fibrinolysis in...
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