Cystic fibrosis and the phenotypic expression of autosomal dominant polycystic kidney disease.

@article{OSullivan1998CysticFA,
  title={Cystic fibrosis and the phenotypic expression of autosomal dominant polycystic kidney disease.},
  author={Denis O'Sullivan and Vicente E. Torres and Patricia A. Gabow and Stephen N. Thibodeau and Bernard F. King and Eric J. Bergstralh},
  journal={American journal of kidney diseases : the official journal of the National Kidney Foundation},
  year={1998},
  volume={32 6},
  pages={976-83}
}
Recent experiments in cultured cyst epithelial cells from kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) have shown that the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is present in the apical surface of these cells and mediates chloride (Cl-) and fluid secretion in vitro. To determine whether the presence of CF with the expression of mutated CFTR proteins modifies cyst formation in ADPKD, we studied a large family with both inherited diseases… CONTINUE READING

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