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

CFTR和TMEM 16 A/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。在这个项目中，我们使用转基因小鼠模型来研究CFTR和TMEM 16 A/F在肾囊肿形成中的作用以及所涉及的信号通路。此外，我们将使用肾脏特异性CFTR、TMEM 16 A/F和SLC 26 A4基因敲除小鼠，探索CFTR、TMEM 16 A和SLC 26 A4（pendrin）在体内代谢代谢过程中的重要性。预期的结果应提供重要的提示，这些氯离子通道在形成肾囊肿和囊性纤维化的代谢性硬化的作用。

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.