Upshaw-Schulman syndrome-associated ADAMTS13 variants possess proteolytic activity at the surface of endothelial cells and in simulated circulation.

Upshaw-Schulman syndrome-associated ADAMTS13 variants possess proteolytic activity at the surface of endothelial cells and in simulated circulation.

ADAMTS13 regulates the hemostatic activity of von Willebrand factor (VWF). Determined by static assays, proteolytic activity <10IU/dL in patient plasma, in absence of ADAMTS13 autoantibodies, indicates Upshaw-Schulman syndrome (USS); the congenital form of Thrombotic Thrombocytopenic Purpura (TTP...

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Bibliographic Details
Main Authors: Anton Letzer, Katja Lehmann, Christian Mess, Gesa König, Tobias Obser, Sven Peine, Sonja Schneppenheim, Ulrich Budde, Stefan W Schneider, Reinhard Schneppenheim, Maria A Brehm
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2020-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0232637

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https://doi.org/10.1371/journal.pone.0232637

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