Characterization of Procoagulant COAT Platelets in Patients with Glanzmann Thrombasthenia.

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License: CC BY 4.0
Serval ID
serval:BIB_80EEBD946701
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Characterization of Procoagulant COAT Platelets in Patients with Glanzmann Thrombasthenia.
Journal
International journal of molecular sciences
Author(s)
Aliotta A., Krüsi M., Bertaggia Calderara D., Zermatten M.G., Gomez F.J., Batista Mesquita Sauvage A.P., Alberio L.
ISSN
1422-0067 (Electronic)
ISSN-L
1422-0067
Publication state
Published
Issued date
14/12/2020
Peer-reviewed
Oui
Volume
21
Number
24
Pages
9515
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Patients affected by the rare Glanzmann thrombasthenia (GT) suffer from defective or low levels of the platelet-associated glycoprotein (GP) IIb/IIIa, which acts as a fibrinogen receptor, and have therefore an impaired ability to aggregate platelets. Because the procoagulant activity is a dichotomous facet of platelet activation, diverging from the aggregation endpoint, we were interested in characterizing the ability to generate procoagulant platelets in GT patients. Therefore, we investigated, by flow cytometry analysis, platelet functions in three GT patients as well as their ability to generate procoagulant collagen-and-thrombin (COAT) platelets upon combined activation with convulxin-plus-thrombin. In addition, we further characterized intracellular ion fluxes during the procoagulant response, using specific probes to monitor by flow cytometry kinetics of cytosolic calcium, sodium, and potassium ion fluxes. GT patients generated higher percentages of procoagulant COAT platelets compared to healthy donors. Moreover, they were able to mobilize higher levels of cytosolic calcium following convulxin-plus-thrombin activation, which is congruent with the greater procoagulant activity. Further investigations will dissect the role of GPIIb/IIIa outside-in signalling possibly implicated in the regulation of platelet procoagulant activity.
Keywords
Glanzmann thrombasthenia, ion fluxes, procoagulant activity, procoagulant platelets
Pubmed
Web of science
Open Access
Yes
Create date
29/12/2020 16:41
Last modification date
12/01/2022 8:11
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