Article
Effects of naturally occurring mutations in CUB-1 domain on synthesis, stability, and activity of ADAMTS-13.
Thrombosis research - 1 Jul 2009
Zhou Zhou, Jing Hua, Tao Zhenyin, Choi Huiwan, Aboulfatova Khatira, Moake Joel, Li Renhao, Dong Jing-Fei
Abstract excerpt
INTRODUCTION: Upon stimulation, endothelial cells release von Willebrand factor (VWF) in the unusually large (UL) and hyperactive forms that are rapidly cleaved by ADAMTS-13. Mutations in the ADAMTS13 gene result in ULVWF-mediated thrombosis found in patients with familial thrombotic thrombocytopenia purpura (TTP). ADAMTS-13 fits in the consensus of the ADAMTS family metalloproteases, but also contains two unique...
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