Article
ADAMTS-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor.
The Journal of biological chemistry - 8 Aug 2003
Dong Jing-fei, Moake Joel L, Bernardo Aubrey, Fujikawa Kazuo, Ball Chalmette, Nolasco Leticia, López José A, Cruz Miguel A
Abstract excerpt
Thrombotic thrombocytopenic purpura is caused by congenital or acquired deficiency of ADAMTS-13, a metalloprotease that cleaves the endothelium-derived ultra-large multimers of von Willebrand factor (ULVWF). The proteolysis converts hyper-reactive and thrombogenic ULVWF into smaller and less adhesive plasma forms. Activity of ADAMTS-13 is usually measured in a static system under non-physiological conditions that...
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