Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs.

@article{Stoltz2013IntestinalCE,
  title={Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs.},
  author={David A. Stoltz and Tatiana Rokhlina and Sarah E. Ernst and Alejandro A. Pezzulo and Lynda S. Ostedgaard and Philip H. Karp and Melissa S. Samuel and Leah R Reznikov and Michael V. Rector and Nicholas D. Gansemer and Drake C. Bouzek and Mahmoud Abou Alaiwa and Mark J. Hoegger and Paula S. Ludwig and Peter J. Taft and Tanner J. Wallen and Christine L. Wohlford-Lenane and James D. McMenimen and Jeng-Haur Chen and Katrina L. Bogan and Ryan Adam and Emma E L Hornick and George A. Nelson and Eric A. Hoffman and Eugene Hanyoung Chang and Joseph Zabner and Paul B. McCray and Randall S Prather and David K. Meyerholz and Michael J. Welsh},
  journal={The Journal of clinical investigation},
  year={2013},
  volume={123 6},
  pages={
          2685-93
        }
}
Cystic fibrosis (CF) pigs develop disease with features remarkably similar to those in people with CF, including exocrine pancreatic destruction, focal biliary cirrhosis, micro-gallbladder, vas deferens loss, airway disease, and meconium ileus. Whereas meconium ileus occurs in 15% of babies with CF, the penetrance is 100% in newborn CF pigs. We hypothesized that transgenic expression of porcine CF transmembrane conductance regulator (pCFTR) cDNA under control of the intestinal fatty acid… CONTINUE READING

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