[New perspectives on the role of αIIbβ3 integrin in defective megakaryopoiesis]

Abstract

In recent years, the understanding of the molecular mechanisms involved in platelet production (megakaryopoiesis) has extremely increased, thanks to the study of genetic diseases causing inherited thrombocytopenia. Among the wide variety of transmembrane receptors covering the platelet membrane, αIIbβ3 integrin is the major one, allowing platelets to aggregate upon the occurrence of vascular breach. Platelet counts are usually normal in patients with αIIbβ3 deficiency, suggesting that its role for normal platelet production and morphology is very limited. However, recently, new clinical observations of genetic diseases provided evidence against this hypothesis, bringing new data on the role of αIIbβ3 integrin in defective megakaryopoiesis.