CFTR and TMEM16A/F in polycystic kidney disease and metabolic alkalosis (A03)

CFTR 和 TMEM16A/F 在多囊肾病和代谢性碱中毒中的作用 (A03)

• 批准号: 415807966
• 金额: --
• 依托单位: Institut für Physiologie
• 依托单位国家: 德国
• 项目类别: Collaborative Research Centres
• 财政年份: 2019
• 资助国家: 德国
• 起止时间: 2018-12-31 至 2021-12-31
• 项目状态: 已结题
• 来源: https://gepris.dfg.de/gepris/projekt/415807966?language=en
• 关键词: CFTR; TMEM16A; polycystic; kidney; disease

CFTR and TMEM16A/F are chloride ion channels that are strongly expressed in the kidney. Their function in the kidney remains poorly understood. In this project, we use genetically modified mouse models to investigate the role of CFTR and TMEM16A/F for renal cyst formation as well as the signaling pathways involved. Moreover, we will explore the importance of CFTR, TMEM16A, and SLC26A4 (pendrin) during metabolic alkalosis in vivo using kidney-specific CFTR, TMEM16A/F, and SLC26A4 knockout mice. The expected results should provide important hints to the role of these Cl- channels in the formation of renal cysts and metabolic alkalosis in cystic fibrosis.

CFTR和TMEM16A/F是在肾脏中强烈表达的氯离子通道。它们在肾脏中的功能仍然知之甚少。在本项目中，我们使用转基因小鼠模型来研究CFTR和TMEM16A/F在肾囊肿形成中的作用及其信号通路。此外，我们将利用肾脏特异性CFTR、TMEM16A/F和SLC26A4敲除小鼠，探讨CFTR、TMEM16A/F和SLC26A4 （penddrin）在体内代谢性碱中毒中的重要性。预期的结果应该为这些Cl-通道在肾囊肿形成和囊性纤维化代谢性碱中毒中的作用提供重要提示。