Article
Force-induced on-rate switching and modulation by mutations in gain-of-function von Willebrand diseases.
Proceedings of the National Academy of Sciences of the United States of America - 14 Apr 2015
Kim Jongseong, Hudson Nathan E, Springer Timothy A
Abstract excerpt
Mutations in the ultralong vascular protein von Willebrand factor (VWF) cause the common human bleeding disorder, von Willebrand disease (VWD). The A1 domain in VWF binds to glycoprotein Ibα (GPIbα) on platelets, in a reaction triggered, in part, by alterations in flow during bleeding. Gain-of-function mutations in A1 and GPIbα in VWD suggest conformational regulation. We report that force application switches A1...
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