The DOI matches its Crossref record; 33 of the 33 references it cites were found in a registry or an index.
Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis
Open accessAbstract
Cystic fibrosis (CF) is a recessive disease that affects multiple organs. It is caused by mutations in CFTR. Animal modeling of this disease has been challenging, with species- and strain-specific differences in organ biology and CFTR function influencing the emergence of disease pathology. Here, we report the phenotype of a CFTR-knockout ferret model of CF. Neonatal CFTR-knockout ferrets demonstrated many of the characteristics of human CF disease, including defective airway chloride transport and submucosal gland fluid secretion; variably penetrant meconium ileus (MI); pancreatic, liver, and vas deferens disease; and a predisposition to lung infection in the early postnatal period. Severe malabsorption by the gastrointestinal (GI) tract was the primary cause of death in CFTR-knockout kits that escaped MI. Elevated liver function tests in CFTR-knockout kits were corrected by oral administration of ursodeoxycholic acid, and the addition of an oral proton-pump inhibitor improved weight gain and survival. To overcome the limitations imposed by the severe intestinal phenotype, we cloned 4 gut-corrected transgenic CFTR-knockout kits that expressed ferret CFTR specifically in the intestine. One clone passed feces normally and demonstrated no detectable ferret CFTR expression in the lung or liver. The animals described in this study are likely to be useful tools for dissecting CF disease pathogenesis and developing treatments.
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Self-citationSubmucosal glands are the predominant site of CFTR expression in the human bronchus
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Self-citationCystic fibrosis genetics: from molecular understanding to clinical application
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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
The AAV Vector Toolkit: Poised at the Clinical Crossroads
Asokan, A., Schaffer, D. V., & Jude Samulski, R. (2012) · Molecular Therapy · doi:10.1038/mt.2011.287 · Cited by 465
Jenkins, R. G., Moore, B. B., Chambers, R. C., Eickelberg, O., Königshoff, M., Kolb, M., Laurent, G. J., Nanthakumar, C. B., Olman, M. A., Pardo, A., et al. (2017) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2017-0096st · Cited by 435
Metadata
- Authors
- Sun, X., Sui, H., Fisher, J. T., Yan, Z., Liu, X., Cho, H.-J., Joo, N. S., Zhang, Y., Zhou, W., Yi, Y., Kinyon, J. M., Lei-Butters, D. C., Griffin, M. A., Naumann, P., Luo, M., Ascher, J., Wang, K., Frana, T., Wine, J. J., Meyerholz, D. K., & Engelhardt, J. F.
- Venue
- Journal of Clinical Investigation
- Published
- 2010-09-01
- Volume
- 120
- Issue
- 9
- Pages
- 3149-3160
- ISSN
- 0021-9738 (ISSN-L)
- OpenAlex
- W1971149849
- Cited by
- 366
- References
- 33
- FWCI
- 21.89
- Type
- journal article
- Language
- en
- Open access
- green
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- Sun, X., Sui, H., Fisher, J. T., Yan, Z., Liu, X., Cho, H.-J., Joo, N. S., Zhang, Y., Zhou, W., Yi, Y., Kinyon, J. M., Lei-Butters, D. C., Griffin, M. A., Naumann, P., Luo, M., Ascher, J., Wang, K., Frana, T., Wine, J. J., . . . Engelhardt, J. F. (2010). Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. Journal of Clinical Investigation. https://doi.org/10.1172/jci43052
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