{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T10:35:54Z","timestamp":1790332554125,"version":"4.1.0"},"reference-count":58,"publisher":"MDPI AG","issue":"24","license":[{"start":{"date-parts":[[2020,12,14]],"date-time":"2020-12-14T00:00:00Z","timestamp":1607904000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100013849","name":"Fondation Dr Henri Dubois-Ferri\u00e8re Dinu Lipatti","doi-asserted-by":"publisher","award":["2017"],"award-info":[{"award-number":["2017"]}],"id":[{"id":"10.13039\/501100013849","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004784","name":"Novartis Stiftung\u00a0f\u00fcr\u00a0Medizinisch-Biologische Forschung","doi-asserted-by":"publisher","award":["18B074"],"award-info":[{"award-number":["18B074"]}],"id":[{"id":"10.13039\/501100004784","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004362","name":"Schweizerische Herzstiftung","doi-asserted-by":"publisher","award":["FF19117"],"award-info":[{"award-number":["FF19117"]}],"id":[{"id":"10.13039\/501100004362","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001711","name":"Schweizerischer Nationalfonds zur F\u00f6rderung der Wissenschaftlichen Forschung","doi-asserted-by":"publisher","award":["320030-197392"],"award-info":[{"award-number":["320030-197392"]}],"id":[{"id":"10.13039\/501100001711","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3390\/ijms21249515","type":"journal-article","created":{"date-parts":[[2020,12,14]],"date-time":"2020-12-14T21:25:08Z","timestamp":1607981108000},"page":"9515","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Characterization of Procoagulant COAT Platelets in Patients with Glanzmann Thrombasthenia"],"prefix":"10.3390","volume":"21","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9382-138X","authenticated-orcid":false,"given":"Alessandro","family":"Aliotta","sequence":"first","affiliation":[{"name":"Division of Hematology and Central Hematology Laboratory, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, CH-1011 Lausanne, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuel","family":"Kr\u00fcsi","sequence":"additional","affiliation":[{"name":"Division of Hematology and Central Hematology Laboratory, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, CH-1011 Lausanne, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9756-4974","authenticated-orcid":false,"given":"Debora","family":"Bertaggia Calderara","sequence":"additional","affiliation":[{"name":"Division of Hematology and Central Hematology Laboratory, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, CH-1011 Lausanne, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxime G.","family":"Zermatten","sequence":"additional","affiliation":[{"name":"Division of Hematology and Central Hematology Laboratory, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, CH-1011 Lausanne, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisco J.","family":"Gomez","sequence":"additional","affiliation":[{"name":"Division of Hematology and Central Hematology Laboratory, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, CH-1011 Lausanne, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana P.","family":"Batista Mesquita Sauvage","sequence":"additional","affiliation":[{"name":"Division of Hematology and Central Hematology Laboratory, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, CH-1011 Lausanne, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9686-9920","authenticated-orcid":false,"given":"Lorenzo","family":"Alberio","sequence":"additional","affiliation":[{"name":"Division of Hematology and Central Hematology Laboratory, Lausanne University Hospital (CHUV) and University of Lausanne (UNIL), Rue du Bugnon 46, CH-1011 Lausanne, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,12,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/j.thromres.2011.11.036","article-title":"Platelets and primary haemostasis","volume":"129","author":"Clemetson","year":"2012","journal-title":"Thromb. 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