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Survival protein anoctamin‐6 controls multiple platelet responses including phospholipid scrambling, swelling, and protein cleavage

Mattheij, Nadine J. A. ; Braun, Attila ; Kruchten, Roger ; Castoldi, Elisabetta ; Pircher, Joachim ; Baaten, Constance C. F. M. J. ; Wülling, Manuela ; Kuijpers, Marijke J. E. ; Köhler, Ralf ; Poole, Alastair W. ; Schreiber, Rainer ; Vortkamp, Andrea ; Collins, Peter W. ; Nieswandt, Bernhard ; Kunzelmann, Karl ; Cosemans, Judith M. E. M ; Heemskerk, Johan W. M.



Abstract

Scott syndrome is a rare bleeding disorder, characterized by altered Ca2+-dependent platelet signaling with defective phosphatidylserine (PS) exposure and microparticle formation, and is linked to mutations in the ANO6 gene, encoding anoctamin (Ano) 6. We investigated how the complex platelet phenotype of this syndrome is linked to defective expression of Anos or other ion channels. Mice were ...

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