Article
Impact of mutations in the von Willebrand factor A2 domain on ADAMTS13-dependent proteolysis.
Blood - 15 Mar 2006
Hassenpflug Wolf Achim, Budde Ulrich, Obser Tobias, Angerhaus Dorothea, Drewke Elke, Schneppenheim Sonja, Schneppenheim Reinhard
Abstract excerpt
Classical von Willebrand disease (VWD) type 2A, the most common qualitative defect of VWD, is caused by loss of high-molecular-weight multimers (HMWMs) of von Willebrand factor (VWF). Underlying mutations cluster in the A2 domain of VWF around its cleavage site for ADAMTS13. We investigated the impact of mutations commonly found in patients with VWD type 2A on ADAMTS13-dependent proteolysis of VWF. We used...
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