THE BIOLOGY OF SICKLE CELL DISEASE

镰状细胞病的生物学

• 批准号: 3098178
• 负责人: EUGENE GOLDWASSER
• 金额: $ 94.11万
• 依托单位: UNIVERSITY OF CHICAGO
• 依托单位国家: 美国
• 财政年份: 1988
• 资助国家: 美国
• 起止时间: 1988-12-01 至 1993-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/3098178
• 关键词: hemoglobin Ss; hemoprotein biosynthesis; hemoprotein structure; molecular pathology; sickle cell anemia

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.

芝加哥大学的几个独立科学家组成 试图在镰状细胞疾病研究中创建一个协调的计划。 他们的方法差异很大，但他们具有共同的兴趣： 健康和疾病中的红细胞，尤其是镰状细胞疾病。 他们的目的是围绕镰状细胞的话题更加仔细 疾病;创建一个高度互动的科学小组，其多样 利益将是协同作用的；为了培养新的，更好的研究，既 个人与协作；并通过 协调的科学信息，仪器，核心共享 设施，患者和数据。 计划项目的好处将 促进共同教育，新的科学见解，改善 合作，以及通过 核心细胞疾病的中心重点。 本申请寻求支持 这个程序。 该程序的主题是红细胞的生物学 在镰状细胞疾病中。 这项研究源自独立 科学计划，它不是统一的，而是多元化和互补的。 这 因此，程序从成员的广度中汲取了力量， 专业知识范围。 然而，在 研究范围。 Huttenlocher试图减轻闭塞 镰刀流变学造成的脑血管疾病 细胞。 Shen提议阐明 不可逆地插的细胞；变化的亚膜丝状网络。 约瑟夫斯将继续他对血红蛋白的纤维的特征 分辨率电子显微镜。 Potel将将计算机技术应用于 Josephs的EM数据和Huttenlocher的正电子发射的分析 断层扫描。 Steck将检验一个新的假设，该假设解释和承诺 扭转镰状细胞膜的被动渗透性增加 小溶质。 Goldwasser将研究球蛋白基因的表达和 Gross将研究其翻译的控制。 并非所有项目 与所有其他人互动；这将是研究的不切实际目标 源于调查人员而不是一个单一的程序 组织者。 但是，有一个互相兴趣的斯通元素是 有必要帮助提高每个子标题的科学。