Protection against enteric pathogens may partly explain the proposed heterozygote advantage of cystic fibrosis mutations in certain populations.
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References
- 1
Rommens, J.M. et al. Identification of the cystic fibrosis gene: chromosome walking and jumping. Science 245, 1059–1072 (1989).
- 2
Pier, G.B. et al. Salmonella typhi uses CFTR to enter intestinal epithelial cells. Nature 393, 79–82 (1998).
- 3
Saiman, L. & Prince, A. Pseudomonas aeruginosa pili bind to asialoGM1 which is increased on the surface of cystic fibrosis epithelial cells. J. Clin. Invest. 92, 1875–1880 (1993).
- 4
Pier, G.B., Grout, M. & Zaidi, T.S. Cystic fibrosis transmembrane conductance regulator is an epithelial cell receptor for clearance of Pseudomonas aeruginosa from the lung. Proc. Natl. Acad. Sci. USA 4, 12088–12093 (1997).
- 5
Smith, J.J., Travis, S.M., Greenberg, E.P. & Welsh, M.J. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell 85, 229–236 (1996).
- 6
Trezise, A.E. & Buchwald, M. In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator. Nature 353, 434–435. (1991).
- 7
Ameen, N.A., Ardito, T., Kashgarian, M. & Marino, C.R. A unique subset of rat and human intestinal villus cells express the cystic fibrosis transmembrane conductance regulator. Gastroenterol. 108, 1016–1023.(1995).
- 8
Jones, B.D. & Falkow, S. Salmonellosis: host immune responses and bacterial virulence determinants. Annu. Rev. Immunol. 14, 533–561 (1996).
- 9
Weinstein, D.L., O'Neill, B.L., Hone, D.M. & Metcalf, E.S. Differential early interactions between Salmonella enterica serovar typhi and two other pathogenic Salmonella serovars with intestinal epithelial cells. Infect. Immun. 66, 2310–2318 (1998).
- 10
Gabriel, S.E., Brigman, K.N., Koller, B.H., Boucher, R.C. & Stutts, M.J. Cystic fibrosis heterozygote resistance to cholera toxin in the cystic fibrosis mouse model. Science 266, 107–109 (1994).
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Prince, A. The CFTR advantae-capitalizing on a quirk of fate. Nat Med 4, 663–664 (1998) doi:10.1038/nm0698-663
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