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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Detalles Bibliográficos
Autores principales: Letzer, Anton, Lehmann, Katja, Mess, Christian, König, Gesa, Obser, Tobias, Peine, Sven, Schneppenheim, Sonja, Budde, Ulrich, Schneider, Stefan W., Schneppenheim, Reinhard, Brehm, Maria A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2020
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7197795/
https://www.ncbi.nlm.nih.gov/pubmed/32365113
http://dx.doi.org/10.1371/journal.pone.0232637

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