UAB Recessive PKD Research and Translational Core Center

UAB 隐性 PKD 研究与转化核心中心

• 批准号: 7035942
• 负责人: Lisa M Guay-Woodford
• 金额: $ 113.3万
• 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/7035942
• 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：(申请人提供) 阿拉巴马大学伯明翰分校(UAB)隐性多囊肾病核心中心(DAB RPKDCC)将在伯明翰大学建立一个跨学科的PKD相关研究卓越中心，重点是隐性PKD。UAB RPKDCC建立在UAB研究人员核心干部的基础上，建立了专注于人类ARPKD和/或隐性PKD小鼠模型的研究的合作。核心中心扩大到包括来自UAB(机构研究基地)的杰出研究人员，以及美国和加拿大的多个机构(扩展研究基地)。聚集在一起的研究人员的集体专业知识被组织成四个主题组：1)纤毛相关生物学，包括钙介导的机械转导通路；2)上皮运输通路的调节；3)在上皮分化中起关键作用的信号通路；以及4)基质生物学与纤维化。这些重点区域包含了隐性PKD发病机制中的主要机制。核心中心调查人员将受益于获得一套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，使四个专题领域的创新调查成为可能。建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-工程小鼠资源；3)核心C-细胞生理学资源；4)核心D-组织特征和免疫反应剂资源；以及5)核心E-蛋白质组资源。总而言之，这些核心和为该中心聚集的非凡的研究人员队伍将提供对隐性PKD创新和富有成效的研究至关重要的专业知识的广度和深度。凭借其包括临床和基础研究人员的扩展研究基础，这种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。