Article
ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage.
Blood - 1 Nov 2003
Soejima Kenji, Matsumoto Masanori, Kokame Koichi, Yagi Hideo, Ishizashi Hiromichi, Maeda Hiroaki, Nozaki Chikateru, Miyata Toshiyuki, Fujimura Yoshihiro, Nakagaki Tomohiro
Abstract excerpt
A severe lack of von Willebrand factor-cleaving protease (VWF-CP) activity can cause thrombotic thrombocytopenic purpura (TTP). This protease was recently identified as a member of the ADAMTS family, ADAMTS-13. It consists of a preproregion, a metalloprotease domain, a disintegrin-like domain, a thrombospondin type-1 motif (Tsp1), a cysteine-rich domain, a spacer domain, additional Tsp1 repeats, and CUB domains....
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