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ADAMTS13 and von Willebrand Factor in Thrombotic Thrombocytopenic Purpura.
Abstract 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 gene or autoantibodies against ADAMTS13 causes hereditary or acquired (idiopathic) TTP. ADAMTS13 activity is usually normal or modestly reduced (>20%) in other forms of thrombotic microangi...
Source: Annual Review of Medicine - January 14, 2015 Category: Journals (General) Authors: Zheng XL Tags: Annu Rev Med Source Type: research