Kansas Interdisciplinary Center for PKD Research

整体中心描述(由申请人提供)： 堪萨斯PKD研究跨学科中心(堪萨斯PKD中心)的成立是为了支持极具价值的新研究计划，以探索多囊肾病(PKD)的基本机制；提供机会，将新的研究人员引入PKD研究；并在动态、丰富和互动的环境中培养更多跨学科研究的机会。堪萨斯PKD中心的长期目标是找到一种治疗PKD的方法，以减轻患者和受疾病影响的家庭的痛苦。堪萨斯PKD中心研究项目的中心主题是“多囊肾病中的细胞增殖”。之所以选择这个主题，是因为中心的研究人员已经能够确定PKD中的细胞增殖缺陷涉及循环AMP和钙依赖的机制。重点将放在使用小鼠作为模式生物，以及人类PKD囊细胞上。堪萨斯PKD中心由八个部分组成。行政核心将为中心调查人员提供行政支持。生物材料研究核心将建立和维护一个用于PKD研究的人和动物生物材料储存库，包括原代培养的细胞和组织，以及ADPKD、ARPKD和正常人类肾脏组织的DNA和RNA。该中心还将建立和维护携带独特PKD基因突变的小鼠的永生化细胞系，并将为所有中心研究人员提供细胞培养方法学方面的技术援助和培训。将有四个生物医学研究项目：项目1，“依赖cAMP的增殖的钙调节”，项目2，“多囊蛋白-1介导的钙和cAMP信号”，项目3，“氧化应激在PKD进展中的作用”，和项目4，“Cux-1与PKD中的细胞周期调节”。

DESCRIPTION OF OVERALL CENTER (provided by applicant): The Kansas Interdisciplinary Center for PKD Research (Kansas PKD Center) was established to provide support for highly meritorious new research programs to explore the basic mechanisms causing polycystic kidney disease (PKD); to provide opportunities to bring new investigators into PKD research; and to foster opportunities for more interdisciplinary research in a dynamic, enriching, and interactive setting. The long term goal for the Kansas PKD Center is to find a treatment for PKD that will alleviate the suffering of patients and families affected by the disease. The central theme of the research projects in the Kansas PKD Center is "cell proliferation in polycystic kidney disease." This theme was chosen because Center investigators have been able to identify the cell proliferation defect in PKD as one that involves a cyclic AMP- and calcium-dependent mechanism. The focus will be on using the mouse as a model organism, and on human PKD cyst cells. The Kansas PKD Center is comprised of eight components. The Administrative Core will provide administrative support for Center investigators. The Biomaterials Research Core will establish and maintain a repository of human and animal biological materials for PKD research including primary cultured cells and tissues, and DMA and RNA from ADPKD, ARPKD, and normal human kidney tissues. The Core will also establish and maintain immortalized cell lines from mice carrying unique PKD gene mutations and will provide technical assistance and training in cell culture methodology for all Center investigators. There will be four Biomedical Research Projects: Project 1, "Calcium Regulation of cAMP-Dependent Proliferation," Project 2, "Polycystin-1 Mediated Calcium and cAMP Signaling," Project 3, "Role of Oxidant Stress in Progression of PKD," and Project 4, "Cux-1 and Cell Cycle Regulation in PKD."