The DOI matches its Crossref record; 8 of the 8 references it cites were found in a registry or an index.
Cystic fibrosis: a mucosal immunodeficiency syndrome
Open accessAbstract
Cystic fibrosis transmembrane conductance regulator (CFTR) functions as a channel that regulates the transport of ions and the movement of water across the epithelial barrier. Mutations in CFTR, which form the basis for the clinical manifestations of cystic fibrosis, affect the epithelial innate immune function in the lung, resulting in exaggerated and ineffective airway inflammation that fails to eradicate pulmonary pathogens. Compounding the effects of excessive neutrophil recruitment, the mutant CFTR channel does not transport antioxidants to counteract neutrophil-associated oxidative stress. Whereas mutant CFTR expression in leukocytes outside of the lung does not markedly impair their function, the expected regulation of inflammation in the airways is clearly deficient in cystic fibrosis. The resulting bacterial infections, which are caused by organisms that have substantial genetic and metabolic flexibility, can resist multiple classes of antibiotics and evade phagocytic clearance. The development of animal models that approximate the human pulmonary phenotypes—airway inflammation and spontaneous infection—may provide the much-needed tools to establish how CFTR regulates mucosal immunity and to test directly the effect of pharmacologic potentiation and correction of mutant CFTR function on bacterial clearance.
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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
The Involvement of Cell-to-Cell Signals in the Development of a Bacterial Biofilm
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A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
Ramsey, B. W., Davies, J., McElvaney, N. G., Tullis, E., Bell, S. C., Dřevínek, P., Griese, M., McKone, E. F., Wainwright, C. E., Konstan, M. W., et al. (2011) · New England Journal of Medicine · doi:10.1056/nejmoa1105185 · Cited by 2,195
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Analysis of quorum sensing-deficient clinical isolates of Pseudomonas aeruginosa
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Nuclear Factor-κB p50 Limits Inflammation and Prevents Lung Injury during Escherichia coli Pneumonia
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Cited by
Elborn, J. S. (2016) · The Lancet · doi:10.1016/s0140-6736(16)00576-6 · Cited by 1,805
Pseudomonas aeruginosa : new insights into pathogenesis and host defenses
Gellatly, S. L., & Hancock, R. E. W. (2013) · Pathogens and Disease · doi:10.1111/2049-632x.12033 · Cited by 1,295
Intestinal alkaline phosphatase: novel functions and protective effects
Lallès, J.-P. (2014) · Nutrition Reviews · doi:10.1111/nure.12082 · Cited by 326
Kahl, B. C., Becker, K., & Löffler, B. (2016) · Clinical Microbiology Reviews · doi:10.1128/cmr.00069-15 · Cited by 325
Use of precision cut lung slices as a translational model for the study of lung biology
Liu, G., Betts, C., Cunoosamy, D. M., Åberg, P. M., Hornberg, J. J., Sivars, K. B., & Cohen, T. S. (2019) · Respiratory Research · doi:10.1186/s12931-019-1131-x · Cited by 206
Self-citation
Metadata
- Authors
- Cohen, T. S., & Prince, A.
- Venue
- Nature Medicine
- Published
- 2012-04
- Volume
- 18
- Issue
- 4
- Pages
- 509-519
- ISSN
- 1078-8956 (ISSN-L)
- 1546-170X
- OpenAlex
- W2040233646
- Cited by
- 484
- References
- 8
- FWCI
- 35.57
- Type
- journal article
- Language
- en
- Open access
- green
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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.
