Article
Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity.
Proceedings of the National Academy of Sciences of the United States of America - 3 Sept 2002
Kokame Koichi, Matsumoto Masanori, Soejima Kenji, Yagi Hideo, Ishizashi Hiromichi, Funato Masahisa, Tamai Hiroshi, Konno Mutsuko, Kamide Kei, Kawano Yuhei, Miyata Toshiyuki, Fujimura Yoshihiro
Abstract excerpt
von Willebrand factor (VWF) is synthesized primarily in vascular endothelial cells and secreted into the plasma as unusually large VWF multimers. Normally, these multimers are quickly degraded into smaller forms by a plasma metalloproteinase, VWF-cleaving protease (VWF-CP). Decreases in the activity of this enzyme result in congenital and acquired thrombotic thrombocytopenic purpura (TTP). The human VWF-CP has...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.