BIOLOGY OF SICKLE CELL DISEASE

芝加哥大学的几名独立科学家组成的小组 寻求在镰状细胞疾病研究中创建一个协调的计划。 他们的做法差异很大，但他们有一个共同的利益： 红细胞在健康和疾病中的作用，特别是镰状细胞疾病。 他们的目标是围绕镰状细胞这个话题拉近距离。 疾病；创建一个高度互动的科学小组，其多样化 兴趣将是协同的；为了促进新的和更好的研究，两者 个人和协作；并通过以下方式加快此类研究 协调共享科学信息、仪器、核心 设施、患者和数据。该计划项目的好处将是 被促进相互教育，新的科学见解，提高 协作，并通过 集中关注镰状细胞疾病。此应用程序寻求对以下各项的支持 这个节目。这个节目的主题是红细胞的生物学。 在镰状细胞病中。因为这项研究是由独立的 科学方案，它不是单一的，而是多样的和互补的。这个 因此，该计划从其成员的广度和广泛的 一系列专业知识。然而，有相当大的连续性，在 研究范围。Huttenlocher试图缓解闭塞 由镰状核变性受损引起的脑血管疾病 细胞。沈建国建议阐明该系统的结构基础 不可逆的病态细胞；改变的亚膜丝状网络。 约瑟夫斯将继续他对血红蛋白S纤维的表征 分辨电子显微镜。Potel将把计算机技术应用于 约瑟夫斯电磁数据和Huttenlocher正电子发射的分析 断层摄影术。Steck将测试一种新的假说，它解释并承诺 为了逆转镰状细胞膜被动通透性的增加 小溶质。戈德瓦瑟将研究珠蛋白基因的表达和 格罗斯将研究他们的翻译控制。并非所有项目 与所有其他人互动；这将是一个不切实际的研究目标 由调查人员发起的程序，而不是单个 组织者。然而，有一个共同利益的强烈因素是 势必有助于推动每个子项目的科学研究。

A group of several independent scientists at The University of Chicago seeks to create a coordinated program in sickle cell disease research. Their approaches vary widely, but they share a common interest: the erythrocyte in health and disease, sickle cell disease in particular. Their aims are to draw closer together around the topic of sickle cell disease; to create a highly interactive scientific group whose diverse interests will be synergistic; to foster new and better research, both individual and collaborative; and to expedite such research through coordinated sharing of scientific information, instrumentation, core facilities, patients, and data. The benefits of the program project will be the promotion of mutual education, new scientific insights, improved collaboration, and increased efficiency and economy of research through a central focus on sickle cell disease. This application seeks support for this program. The theme of this program is the biology of the erythrocyte in sickle cell disease. In that the research emanates from independent scientific programs, it is not unitary but diverse and complementary. The program thus draws strength from the breadth of its membership and a wide range of expertise. There is, nevertheless, considerable continuity in the scope of the research. Huttenlocher seeks to relieve the occlusive cerebral vascular disease arising from the compromised rheology of sickled cells. Shen proposes to elucidate the structural basis for the irreversibly-sickled cell; an altered sub-membrane filamentous network. Josephs will continue his characterization of hemoglobin S fibers by high resolution electron microscopy. Potel will apply computer techniques to the analysis of Josephs's EM data and Huttenlocher's positron emission tomographs. Steck will test a novel hypothesis which explains and promises to reverse the increased passive permeability of sickle cell membranes to small solutes. Goldwasser will study the expression of globin genes and Gross will study the control of their translation. Not all projects interact with all others; this would be an unrealistic goal for research programs that originate with investigators rather than with a single organizer. There is, however, a stong element of mutual interest that is bound to help advance the science in each subproject.