NURDEN, A. T.; PILLOIS, X.

doi:10.1080/09537104.2017.1371291

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NURDEN, A. T.

PILLOIS, X.
Biologie des maladies cardiovasculaires = Biology of Cardiovascular Diseases

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Platelets. 2018-01, vol. 29(1), n° 98-101

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Studies on Glanzmann thrombasthenia (GT) helped discover the αIIbβ3 integrin and led to successful antithrombotic therapy [1,2]. GT is defined by a lack of platelet aggregation with physiological agonists, but a normal response to ristocetin. Historically, patients have been segregated into three groups: type I where platelets lack αIIbβ3 (by definition \textless5% of the normal platelet content), type II with residual αIIbβ3 (usually 5–15%) that may be functional or not, while in type III or variant GT αIIbβ3 fails to function despite platelets having up to 100% of normal levels [3]. αIIbβ3 assures platelet aggregation by changing from a bent to an extended and open form on platelet activation, conformational changes that allow fibrinogen (Fg) or other adhesive proteins to bind and crosslink platelets [4]. The absence of this function causes severe mucocutaneous bleeding and perhaps underestimated nonhemostatic defects often linked to loss of the wider biological expression of αvβ3 [5]. Inherited thrombocytopenia and increased platelet size associate with a GT-like phenotype in rare families [6]. ...< Réduire

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ITGA2B and ITGB3 gene mutations associated with Glanzmann thrombasthenia

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