Article
Genetic alteration of the D2 domain abolishes von Willebrand factor multimerization and trafficking into storage.
Journal of thrombosis and haemostasis : JTH - 1 Apr 2009
Haberichter S L, Allmann A M, Jozwiak M A, Montgomery R R, Gill J C
Abstract excerpt
BACKGROUND: The large von Willebrand factor (VWF) propeptide (VWFpp) plays a critical role in the multimerization and regulated storage of the mature VWF protein. Although our laboratory and others have identified mutations in von Willebrand disease patients that disrupt VWF multimerization, little is known about the affect of mutations on the regulated storage of VWF. PATIENTS/METHODS: We identified a...
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