Oxidative stress on VWF proteolysis.

摘要

Von Willebrand factor (VWF) multimers secreted from Weibel-Palade bodies of endothelial cells are enriched in ultra-large forms that are rapidly cleaved by the zinc met-alloprotease AD AMTS13. Enzyme deficiency is associated with the development of throm-botic thrombocytopenic purpura as well as with conditions associated with systemic inflammation. There have been extensive studies on how VWF proteolysis is initiated and regulated, mostly focusing on identifying the interface between the substrate and metallo-protease, and specific mutations that alter the rate of cleavage.