Carubbi Cecilia, Masselli Elena, Martini Silvia, Galli Daniela, Aversa Franco, Mirandola Prisco, Italiano Joseph E, Gobbi Giuliana, Vitale Marco
Department of Biomedical, Biotechnological and Translational Sciences (SBiBiT), University of Parma, Italy.
Department of Clinical and Experimental Medicine, University of Parma, Italy.
Haematologica. 2016 Jul;101(7):812-20. doi: 10.3324/haematol.2015.137984. Epub 2016 Apr 14.
A deeper understanding of the molecular events driving megakaryocytopoiesis and thrombopoiesis is essential to regulate in vitro and in vivo platelet production for clinical applications. We previously documented the crucial role of PKCε in the regulation of human and mouse megakaryocyte maturation and platelet release. However, since several data show that different PKC isoforms fulfill complementary functions, we targeted PKCε and PKCδ, which show functional and phenotypical reciprocity, at the same time as boosting platelet production in vitro. Results show that PKCδ, contrary to PKCε, is persistently expressed during megakaryocytic differentiation, and a forced PKCδ down-modulation impairs megakaryocyte maturation and platelet production. PKCδ and PKCε work as a functional couple with opposite roles on thrombopoiesis, and the modulation of their balance strongly impacts platelet production. Indeed, we show an imbalance of PKCδ/PKCε ratio both in primary myelofibrosis and essential thrombocythemia, featured by impaired megakaryocyte differentiation and increased platelet production, respectively. Finally, we demonstrate that concurrent molecular targeting of both PKCδ and PKCε represents a strategy for in vitro platelet factories.
深入了解驱动巨核细胞生成和血小板生成的分子事件对于调节体外和体内血小板生成以用于临床应用至关重要。我们之前记录了PKCε在调节人和小鼠巨核细胞成熟及血小板释放中的关键作用。然而,由于多项数据表明不同的PKC亚型具有互补功能,我们在体外促进血小板生成的同时,针对具有功能和表型互补性的PKCε和PKCδ进行研究。结果显示,与PKCε相反,PKCδ在巨核细胞分化过程中持续表达,强制下调PKCδ会损害巨核细胞成熟和血小板生成。PKCδ和PKCε在血小板生成中作为一对功能相反的伴侣发挥作用,调节它们之间的平衡会强烈影响血小板生成。事实上,我们发现原发性骨髓纤维化和原发性血小板增多症中PKCδ/PKCε比值均失衡,分别表现为巨核细胞分化受损和血小板生成增加。最后,我们证明同时对PKCδ和PKCε进行分子靶向是体外血小板工厂的一种策略。