Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs.

doi:10.1172/JCI68867

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Article

Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs.

The Journal of clinical investigation - 1 Jun 2013

Stoltz David A, Rokhlina Tatiana, Ernst Sarah E, Pezzulo Alejandro A, Ostedgaard Lynda S, Karp Philip H, Samuel Melissa S, Reznikov Leah R, Rector Michael V, Gansemer Nicholas D, Bouzek Drake C, Abou Alaiwa Mahmoud H, Hoegger Mark J, Ludwig Paula S, Taft Peter J, Wallen Tanner J, Wohlford-Lenane Christine, McMenimen James D, Chen Jeng-Haur, Bogan Katrina L, Adam Ryan J, Hornick Emma E, Nelson George A, Hoffman Eric A, Chang Eugene H, Zabner Joseph, McCray Paul B, Prather Randall S, Meyerholz David K, Welsh Michael J

Abstract excerpt

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...

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PMID: 23676501
DOI: 10.1172/JCI68867
PMCID: PMC3668832

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CFTR

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