The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs
Open accessAbstract
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.
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Cites
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
Riordan, J. R., Rommens, J. M., Kerem, B.-S., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J.-L., et al. (1989) · Science · doi:10.1126/science.2475911 · Cited by 7,418
Cited by
The gastrointestinal mucus system in health and disease
Johansson, M. E. V., Sjövall, H., & Hansson, G. C. (2013) · Nature Reviews Gastroenterology & Hepatology · doi:10.1038/nrgastro.2013.35 · Cited by 1,438
O'Sullivan, B. P., & Freedman, S. D. (2009) · The Lancet · doi:10.1016/s0140-6736(09)60327-5 · Cited by 1,320
Cystic fibrosis genetics: from molecular understanding to clinical application
Cutting, G. R. (2015) · Nature Reviews Genetics · doi:10.1038/nrg3849 · Cited by 965
Generation of transgenic non-human primates with germline transmission
Sasaki, E., Suemizu, H., Shimada, A., Hanazawa, K., Oiwa, R., Kamioka, M., Tomioka, I., Sotomaru, Y., Hirakawa, R., Eto, T., et al. (2009) · Nature · doi:10.1038/nature08090 · Cited by 712
Molecular Structure of the Human CFTR Ion Channel
Liu, F., Zhang, Z., Csanády, L., Gadsby, D. C., & Chen, J. (2017) · Cell · doi:10.1016/j.cell.2017.02.024 · Cited by 523
Metadata
- Authors
- Rogers, C. S., Stoltz, D. A., Meyerholz, D. K., Ostedgaard, L. S., Rokhlina, T., Taft, P. J., Rogan, M. P., Pezzulo, A. A., Karp, P. H., Itani, O. A., Kabel, A. C., Wohlford-Lenane, C. L., Davis, G. J., Hanfland, R. A., Smith, T. L., Samuel, M., Wax, D., Murphy, C. N., Rieke, A., Whitworth, K., Uc, A., Starner, T. D., Brogden, K. A., Shilyansky, J., McCray, P. B., Zabner, J., Prather, R. S., & Welsh, M. J.
- Venue
- Science
- Published
- 2008-09-26
- Volume
- 321
- Issue
- 5897
- Pages
- 1837-1841
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2087326716
- Cited by
- 749
- References
- 1
- FWCI
- 45.35
- Type
- journal article
- Language
- en
- Open access
- green
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- The paper
- Rogers, C. S., Stoltz, D. A., Meyerholz, D. K., Ostedgaard, L. S., Rokhlina, T., Taft, P. J., Rogan, M. P., Pezzulo, A. A., Karp, P. H., Itani, O. A., Kabel, A. C., Wohlford-Lenane, C. L., Davis, G. J., Hanfland, R. A., Smith, T. L., Samuel, M., Wax, D., Murphy, C. N., Rieke, A., . . . Welsh, M. J. (2008). Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs. Science. https://doi.org/10.1126/science.1163600
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
