UAB Recessive PKD Research and Translational Core Center

UAB 隐性 PKD 研究与转化核心中心

• 批准号: 7127320
• 负责人: Lisa M Guay-Woodford
• 金额: $ 105.51万
• 依托单位: UNIVERSITY OF ALABAMA AT BIRMINGHAM
• 依托单位国家: 美国
• 财政年份: 2005
• 资助国家: 美国
• 起止时间: 2005-09-30 至 2010-08-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7127320
• 关键词: autosomal recessive trait; clinical research; polycystic kidney

DESCRIPTION: (provided by applicant) The University of Alabama at Birmingham (UAB) Recessive Polycystic Kidney Disease Core Center (DAB RPKDCC) will establish a UAB-based interdisciplinary center of excellence in PKD-related research, with specific emphasis on recessive PKD. The UAB RPKDCC builds on a core cadre of UAB investigators with established collaborations focused on investigations of human ARPKD and/or mouse models of recessive PKD. The Core Center expands to include outstanding investigators from UAB (Institutional Research Base), as well as multiple institutions in the US and Canada (Extended Research Base). The collective expertise of the assembled investigators has been organized into four thematic groups: 1) cilia-related biology, including calcium-mediated mechanotransduction pathways; 2) regulation of epithelial transport pathways; 3) signaling pathways that are critical in epithelial differentiation; and 4) matrix biology and fibrosis. These areas of emphasis incorporate the major mechanisms implicated in the pathogenesis of recessive PKD. The Core Center investigators will benefit from access to a set of five complementary Biomedical Research Cores that will integrate existing intellectual and technological resources of the University and provide a set of services/resources that will enable innovative investigations in the four thematic areas. The proposed biomedical cores are: 1) Core A - The ARPKD Clinical and Genetic Resource; 2) Core B - The Engineered Mouse Resource; 3) Core C - The Cellular Physiology Resource; 4) Core D - The Tissue Characterization and Immunoreagent Resource; and 5) Core E - The Proteomic Resource. Taken together, these Cores and the extraordinary cohort of investigators assembled for this Center will provide the breadth and depth of expertise that is critical for innovative and productive research in recessive PKD. With its Extended Research Base that includes both clinical and basic investigators, this Core Center model will accelerate the translation of new investigative insights toward new therapies for ARPKD patients.

描述：（由申请人提供） 阿拉巴马大学伯明翰分校 (UAB) 隐性多囊肾核心中心 (DAB RPKDCC) 将建立一个基于 UAB 的 PKD 相关研究跨学科卓越中心，特别关注隐性多囊肾。 UAB RPKDCC 建立在 UAB 研究人员的核心骨干基础上，并建立了专注于人类 ARPKD 和/或隐性 PKD 小鼠模型研究的合作。核心中心扩大到包括来自UAB（机构研究基地）以及美国和加拿大多个机构（扩展研究基地）的优秀研究人员。聚集的研究人员的集体专业知识被分为四个主题组：1）纤毛相关生物学，包括钙介导的机械转导途径； 2）上皮运输途径的调节； 3）对上皮分化至关重要的信号通路； 4）基质生物学和纤维化。这些重点领域包括隐性 PKD 发病机制中涉及的主要机制。核心中心的研究人员将受益于一组五个互补的生物医学研究核心，这些核心将整合大学现有的智力和技术资源，并提供一套服务/资源，以便在四个主题领域进行创新研究。拟议的生物医学核心是： 1) 核心 A - ARPKD 临床和遗传资源； 2) 核心 B - 工程鼠标资源； 3) 核心C——细胞生理学资源； 4) 核心 D——组织表征和免疫试剂资源； 5) 核心 E - 蛋白质组学资源。总而言之，这些核心人员和为该中心聚集的杰出研究人员群体将提供广度和深度的专业知识，这对于隐性 PKD 的创新和富有成效的研究至关重要。凭借其包括临床和基础研究人员的扩展研究基地，该核心中心模式将加速将新的研究见解转化为 ARPKD 患者的新疗法。