Article
ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura.
Annual review of medicine - 1 Jan 2015
Zheng X Long
Abstract excerpt
Pathogenesis of thrombotic thrombocytopenic purpura (TTP) was a mystery for over half a century until the discovery of ADAMTS13. ADAMTS13 is primarily synthesized in the liver, and its main function is to cleave von Willebrand factor (VWF) anchored on the endothelial surface, in circulation, and at the sites of vascular injury. Deficiency of plasma ADAMTS13 activity (<10%) resulting from mutations of the ADAMTS13...
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