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Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs.
- Source :
-
Science (New York, N.Y.) [Science] 2008 Sep 26; Vol. 321 (5897), pp. 1837-41. - Publication Year :
- 2008
-
Abstract
- Almost two decades after CFTR was identified as the gene responsible for cystic fibrosis (CF), we still lack answers to many questions about the pathogenesis of the disease, and it remains incurable. Mice with a disrupted CFTR gene have greatly facilitated CF studies, but the mutant mice do not develop the characteristic manifestations of human CF, including abnormalities of the pancreas, lung, intestine, liver, and other organs. Because pigs share many anatomical and physiological features with humans, we generated pigs with a targeted disruption of both CFTR alleles. Newborn pigs lacking CFTR exhibited defective chloride transport and developed meconium ileus, exocrine pancreatic destruction, and focal biliary cirrhosis, replicating abnormalities seen in newborn humans with CF. The pig model may provide opportunities to address persistent questions about CF pathogenesis and accelerate discovery of strategies for prevention and treatment.
- Subjects :
- Animals
Animals, Newborn
Chlorides metabolism
Cystic Fibrosis Transmembrane Conductance Regulator metabolism
Female
Gallbladder pathology
Ileus pathology
Ileus physiopathology
Intestines pathology
Ion Transport
Liver pathology
Liver Cirrhosis, Biliary pathology
Lung pathology
Male
Pancreas, Exocrine pathology
Recombination, Genetic
Cystic Fibrosis genetics
Cystic Fibrosis pathology
Cystic Fibrosis physiopathology
Cystic Fibrosis Transmembrane Conductance Regulator genetics
Disease Models, Animal
Swine
Subjects
Details
- Language :
- English
- ISSN :
- 1095-9203
- Volume :
- 321
- Issue :
- 5897
- Database :
- MEDLINE
- Journal :
- Science (New York, N.Y.)
- Publication Type :
- Academic Journal
- Accession number :
- 18818360
- Full Text :
- https://doi.org/10.1126/science.1163600