Document Detail

Phosphatidylserine exposure and other apoptotic-like events in Bernard-Soulier syndrome platelets.
MedLine Citation:
PMID:  20658588     Owner:  NLM     Status:  MEDLINE    
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 DeltaPsi(m) loss, but unlike controls, there was also loss of DeltaPsi(m) in the BSS platelets not exposing PS. Thus, BSS platelets undergo apoptotic-like events upon activation, with PS exposure and MP formation being enhanced. These events may play a role in the shortened survival in BSS, as well as affecting thrombin generation.
Margaret L Rand; Hong Wang; K W Annie Bang; Jerome M Teitel; Victor S Blanchette; John Freedman; Alan T Nurden
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American journal of hematology     Volume:  85     ISSN:  1096-8652     ISO Abbreviation:  Am. J. Hematol.     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-07-26     Completed Date:  2010-08-11     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7610369     Medline TA:  Am J Hematol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  584-92     Citation Subset:  IM    
Copyright Information:
(c) 2010 Wiley-Liss, Inc.
Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Canada.
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MeSH Terms
Bernard-Soulier Syndrome / blood*
Blood Platelets / chemistry,  drug effects,  pathology*
Cell Size
Collagen / pharmacology
Membrane Glycoproteins / deficiency,  genetics
Membrane Lipids / blood*
Oligopeptides / biosynthesis,  pharmacology
Phosphatidylserines / blood*
Platelet Activation / drug effects
Platelet Count
Platelet Glycoprotein GPIb-IX Complex / genetics
Receptor, PAR-1 / drug effects
Receptors, Thrombin / biosynthesis,  drug effects
Reg. No./Substance:
0/Membrane Glycoproteins; 0/Membrane Lipids; 0/Oligopeptides; 0/PAR-1-activating peptide; 0/Phosphatidylserines; 0/Platelet Glycoprotein GPIb-IX Complex; 0/Receptor, PAR-1; 0/Receptors, Thrombin; 0/adhesion receptor; 0/protease-activated receptor 4; 9007-34-5/Collagen

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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