Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited kidney disease. Transepithelial fluid secretion is one of the key factors of cystogenesis in ADPKD. Multiple studies have suggested that fluid secretion across ADPKD cyst-lining cells is driven by the secretion of chloride, essentially mediated by the CFTR channel and stimulated by increased intracellular levels of 3′,5′-cyclic adenosine monophosphate. This review focuses on the pathophysiology of fluid secretion in ADPKD based on the pioneering studies of Jared Grantham and colleagues, and on the follow-up investigations from the molecular level to the potential applications in ADPKD patients. Altogether, the studies of fluid and chloride transport in ADPKD paved the way for innovative therapeutic targets to prevent cyst volume expansion and thus, kidney disease progression.

常染色体显性多囊肾病（ADPKD）是最常见的遗传性肾病。跨上皮液体分泌是 ADPKD 囊肿发生的关键因素之一。多项研究表明，通过 ADPKD 囊肿衬里细胞的液体分泌是由氯的分泌驱动的，主要由 CFTR 通道介导，并受到细胞内 3',5'-环磷酸腺苷水平增加的刺激。本综述基于 Jared Grantham 及其同事的开创性研究，重点关注 ADPKD 体液分泌的病理生理学，以及从分子水平到 ADPKD 患者潜在应用的后续研究。总之，对 ADPKD 中液体和氯离子转运的研究为防止囊肿体积扩大的创新治疗靶点铺平了道路，因此，