PPG -Airway Physiology and Pathophysiology in a Porcine CF Model

PPG - 猪 CF 模型中的气道生理学和病理生理学

• 批准号: 7904983
• 负责人: MICHAEL J. WELSH
• 金额: $ 235.06万
• 依托单位: UNIVERSITY OF IOWA
• 依托单位国家: 美国
• 财政年份: 2008
• 资助国家: 美国
• 起止时间: 2008-09-01 至 2013-07-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7904983
• 关键词: PPG; Airway; Physiology; Pathophysiology; Porcine

DESCRIPTION (provided by applicant): Eighteen years after identification of the CFTR gene we still lack answers to many crucial questions. Controversies surround the pathogenesis of airway disease, current treatments are inadequate, and cystic fibrosis (CF) remains a lethal disease. A major impediment to progress has been lack of a CF animal model other than the mouse. CF mice fail to develop lung disease, the cause of most CF morbidity and mortality. Therefore, we disrupted the CFTR gene in the pig, whose lungs resemble those of humans. In this Program four senior and highly accomplished investigators will seize the unique opportunity to use CFTR -I- pigs to answer key questions about CF lung disease. Together, the four projects will discover how loss of CFTR causes airway epithelial and submucosal gland dysfunction and how that contributes to airway inflammation and infection. The Project Leaders have an outstanding track record of collaboration in CF, and here they sharpen their focus to a common goal. Their research is highly creative and is supported by five cores that provide innovative services and infrastructure. Discoveries from this PPG will accelerate development of novel therapies for patients who suffer from this devastating disease.

描述（由申请人提供）：鉴定CFTR基因的十八年，我们仍然缺乏许多关键问题的答案。争议围绕气道疾病的发病机理，当前治疗不足，囊性纤维化（CF）仍然是致命的疾病。除了小鼠以外，还缺乏CF动物模型的主要障碍。 CF小鼠无法发展为肺部疾病，这是大多数CF发病率和死亡率的原因。因此，我们破坏了猪中的CFTR基因，其肺类似于人类的肺。在该计划中，四名高级和高度成就的调查人员将抓住独特的机会使用CFTR -I -i -i -i -pig回答有关CF肺部疾病的关键问题。这四个项目将共同发现CFTR的损失如何导致气道上皮和粘膜下腺功能障碍以及这如何促进气道炎症和感染。项目负责人在CF中的合作记录出色，在这里他们将重点放在一个共同的目标上。他们的研究具有很高的创造力，并得到了提供创新服务和基础设施的五个核心的支持。该PPG的发现将加速患有这种毁灭性疾病的患者的新疗法。