Article
The co-influence of VWD type 2B/2M mutations in the A1 domain and platelet GPIbα on the rate of cleavage to VWF by ADAMTS13.
Thrombosis research - 1 Nov 2015
Ma Zhenni, Su Jian, Zhang Jingyu, Ling Jing, Yin Jie, Bai Xia, Ruan Changgeng
Abstract excerpt
INTRODUCTION: In plasma, the size of the von Willebrand factor (VWF) multimer is down-regulated by ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). The binding of platelets or glycoprotein (GP) Ibα recombinant fragment to VWF domain A1 may increase the cleavage by ADAMTS13 to VWF. Both type 2B and type 2M von Willebrand disease (VWD) result in bleeding disorders with the...
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