Ware J, Hashimoto Y, Zieger B, Russell S
Roon Center for Arterioscerosis and Thrombosis, Department of Molecular and Experimental Medicine, Scripps Research Institute, La Jolla, CA 92037, USA.
C R Acad Sci III. 1996 Sep;319(9):811-7.
Our objectives are to define and characterize molecular events of megakaryocytopoiesis and platelet production by analyzing the in vivo expression of platelet glycoprotein (GP) receptors. Our studies target the platelet GP Ib-IX-V complex since the congenital absence of the receptor results in the Bernard-Soulier syndrome, a condition linking the expression of the complex to normal platelet morphogenesis. We have previously described the generation of a transgenic mouse colony expressing the alpha-subunit (GP Ib alpha) of the human platelet GP Ib-IX-V complex. These studies established methodologies to manipulate GP Ib-IX-V on the platelet surface and examine unique aspects of hemostasis, megakaryocytopoiesis, platelet structure and platelet release. Our recent studies have defined the genetic elements supporting the megakaryocytic-expression of GP Ib alpha. These results are defining essential cis-acting elements responsible for the expression of GP Ib alpha and are providing insights into molecular events coinciding with the release of normal platelets into the bloodstream.
我们的目标是通过分析血小板糖蛋白（GP）受体的体内表达，来定义和描述巨核细胞生成及血小板产生的分子事件。我们的研究以血小板GP Ib-IX-V复合物为靶点，因为该受体的先天性缺失会导致Bernard-Soulier综合征，这种病症将该复合物的表达与正常血小板形态发生联系起来。我们之前描述过表达人血小板GP Ib-IX-V复合物α亚基（GP Ibα）的转基因小鼠群体的产生。这些研究建立了在血小板表面操纵GP Ib-IX-V的方法，并研究了止血、巨核细胞生成、血小板结构和血小板释放的独特方面。我们最近的研究确定了支持GP Ibα巨核细胞表达的遗传元件。这些结果确定了负责GP Ibα表达的重要顺式作用元件，并为与正常血小板释放到血流中同时发生的分子事件提供了见解。
