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Role of Mutant CFTR in Hypersusceptibility of Cystic Fibrosis Patients to Lung Infections
Open accessAbstract
Cystic fibrosis (CF) patients are hypersusceptible to chronic Pseudomonas aeruginosa lung infections. Cultured human airway epithelial cells expressing the ΔF508 allele of the cystic fibrosis transmembrane conductance regulator (CFTR) were defective in uptake of P. aeruginosa compared with cells expressing the wild-type allele. Pseudomonas aeruginosa lipopolysaccharide (LPS)-core oligosaccharide was identified as the bacterial ligand for epithelial cell ingestion; exogenous oligosaccharide inhibited bacterial ingestion in a neonatal mouse model, resulting in increased amounts of bacteria in the lungs. CFTR may contribute to a host-defense mechanism that is important for clearance of P. aeruginosa from the respiratory tract.
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Cited by
Worlitzsch, D., Tarran, R., Ulrich, M., Schwab, U., Cekici, A., Meyer, K. C., Birrer, P., Bellon, G., Berger, J., Weiss, T., et al. (2002) · Journal of Clinical Investigation · doi:10.1172/jci0213870 · Cited by 932
Self-citationRatjen, F., & Döring, G. (2003) · The Lancet · doi:10.1016/s0140-6736(03)12567-6 · Cited by 885
CFTR Function and Prospects for Therapy
Riordan, J. R. (2008) · Annual Review of Biochemistry · doi:10.1146/annurev.biochem.75.103004.142532 · Cited by 610
Alton, E., Stern, M., Farley, R., Jaffe, A., Chadwick, S., Phillips, J., Davies, J., Smith, S., Browning, J., Davies, M., et al. (1999) · The Lancet · doi:10.1016/s0140-6736(98)06532-5 · Cited by 435
Bais, H. P., Walker, T. S., Schweizer, H. P., & Vivanco, J. M. (2002) · Plant Physiology and Biochemistry · doi:10.1016/s0981-9428(02)01460-2 · Cited by 361
Metadata
- Authors
- Pier, G. B., Grout, M., Zaidi, T. S., Olsen, J. C., Johnson, L. G., Yankaskas, J. R., & Goldberg, J. B.
- Venue
- Science
- Published
- 1996-01-05
- Volume
- 271
- Issue
- 5245
- Pages
- 64-67
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2088587327
- Cited by
- 440
- References
- 0
- FWCI
- 41.77
- Type
- journal article
- Language
- en
- Open access
- green
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- The paper
- Pier, G. B., Grout, M., Zaidi, T. S., Olsen, J. C., Johnson, L. G., Yankaskas, J. R., & Goldberg, J. B. (1996). Role of Mutant CFTR in Hypersusceptibility of Cystic Fibrosis Patients to Lung Infections. Science. https://doi.org/10.1126/science.271.5245.64
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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.
