Role of CFTR in autosomal recessive polycystic kidney disease.

@article{Nakanishi2001RoleOC,
  title={Role of CFTR in autosomal recessive polycystic kidney disease.},
  author={Koichi Nakanishi and William E. Sweeney and Katherine MacRae Dell and Calvin Utley Cotton and Ellis D. Avner},
  journal={Journal of the American Society of Nephrology : JASN},
  year={2001},
  volume={12 4},
  pages={719-25}
}
An extensive body of in vitro data implicates epithelial chloride secretion, mediated through cystic fibrosis transmembrane conductance regulator (CFTR) protein, in generating or maintaining fluid filled cysts in MDCK cells and in human autosomal dominant polycystic kidney disease (ADPKD). In contrast, few studies have addressed the pathophysiology of fluid secretion in cyst formation and enlargement in autosomal recessive polycystic kidney disease (ARPKD). Murine models of targeted disruptions… CONTINUE READING