Phosphatidylserine exposure and other apoptotic-like events in Bernard-Soulier syndrome platelets

American Journal of Hematology
Margaret L RandAlan T Nurden

Abstract

In the Bernard-Soulier syndrome (BSS), the giant platelets are said to have increased phosphatidylserine (PS) surface exposure in the resting state and shortened survival in the circulation. When normal platelets are activated, they undergo many biochemical and morphological changes, some of which are apoptotic. Herein, we investigated apoptotic-like events in BSS platelets upon activation, specifically, PS exposure, microparticle (MP) formation, cell shrinkage, and loss of mitochondrial inner membrane potential (DeltaPsi(m)). Platelets from two unrelated BSS patients were examined in whole blood; agonists used were collagen, thrombin, PAR1- or PAR4-activating peptides (APs), or combinations of collagen with thrombin, and the PAR-APs. Flow cytometry was used to measure PS exposure (annexin A5 binding), platelet-derived MPs (forward scatter; events <0.75 microm size), and DeltaPsi(m) (TMRM fluorescence). PS exposure was increased on resting and activated BSS platelets, and this was independent of the platelet size. MP formation by BSS platelets was generally enhanced. Cell shrinkage occurred on activation to form smaller, PS-exposing platelets in BSS and controls. A proportion of PS-exposing BSS and control platelets exhibited D...Continue Reading

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Citations

Mar 3, 2012·Journal of Thrombosis and Thrombolysis·Armen V GyulkhandanyanValery Leytin
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Sep 4, 2021·Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis·Nanna BrønsSisse R Ostrowski

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