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American Society of Hematology, Blood Advances, 12(2), p. 1417-1428, 2018

DOI: 10.1182/bloodadvances.2017014290

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Protein kinase C signaling dysfunction in von Willebrand disease (p.V1316M) type 2B platelets

Journal article published in 2018 by Caterina Casari, David S. Paul, Sophie Susen, Cécile Lavenu-Bombled, Annie Harroche, Raymond Piatt, Kathryn O. Poe, Robert H. Lee, Marijke Bryckaert ORCID, Olivier D. Christophe ORCID, Peter J. Lenting, Cécile V. Denis ORCID, Wolfgang Bergmeier
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

Key Points In VWD (p.V1316M) type 2B, the VWF–GPIb interaction alters the sustained PKC-mediated pathway of Rap1 activation. VWF/p.V1316M expression in mice causes multiple platelet dysfunctions, including significant ADAM17-independent shedding of GPIbα.