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Schematic representation of vWF multimer size regulation by ADAMTS13. von Willebrand Factor (vWF) is stored in the Weibel-Palade bodies of endothelial cells or in the α-granules of platelets and it is released in an ultra-large form, a long multimeric string. The vWF serves as an adhesion surface to which platelets adhere and aggregate, and form a plug. The regulation of platelets adhesion depends upon cleavage of vWF in different size of multimeric string by ADAMTS13.
