Note to users. If you're seeing this message, it means that your browser cannot find this page's style/presentation instructions -- or possibly that you are using a browser that does not support current Web standards. Find out more about why this message is appearing, and what you can do to make your experience of our site the best it can be.

Subscribe

Sci. Signal., 30 September 2008
Vol. 1, Issue 39, p. ec342
[DOI: 10.1126/scisignal.139ec342]

EDITORS' CHOICE

Medicine Cystic Fibrosis Remodeled

Paula A. Kiberstis

Science, AAAS, Washington, DC 20005, USA

Cystic fibrosis (CF) is caused by mutational disruption of CFTR, a gene encoding an ion channel required for chloride- and bicarbonate-mediated fluid secretion in epithelia and for salt absorption in many organs. Two decades of intense research on CFTR has not yet translated into new clinical therapies, in part because mice--the traditional animal model for human disease research--do not develop the full spectrum of pathologies seen in human CF. To address this problem, Rogers et al. have inactivated the CFTR gene in pigs, an animal that shares many anatomical and physiological features of humans. Newborn pigs lacking CFTR developed many of the gastrointestinal pathologies seen in infants with CF, including intestinal obstruction and abnormalities of the pancreas, liver, and gallbladder, and their nasal epithelia showed defects in chloride transport. These results, while still preliminary, suggest that the pig model may be a valuable tool for testing new therapies for CF.

C. S. Rogers, D. A. Stoltz, D. K. Meyerholz, L. S. Ostedgaard, T. Rokhlina, P. J. Taft, M. P. Rogan, A. A. Pezzulo, P. H. Karp, O. A. Itani, A. C. Kabel, C. L. Wohlford-Lenane, G. J. Davis, R. A. Hanfland, T. L. Smith, M. Samuel, D. Wax, C. N. Murphy, A. Rieke, K. Whitworth, A. Uc, T. D. Starner, K. A. Brogden, J. Shilyansky, P. B. McCray, Jr., J. Zabner, R. S. Prather, M. J. Welsh, Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science 321, 1837-1841 (2008). [Abstract] [Full Text]

Citation: P. A. Kiberstis, Cystic Fibrosis Remodeled. Sci. Signal. 1, ec342 (2008).


To Advertise     Find Products


Science Signaling. ISSN 1937-9145 (online), 1945-0877 (print). Pre-2008: Science's STKE. ISSN 1525-8882