The DOI matches its Crossref record; 21 of the 21 references it cites were found in a registry or an index.
Cystic fibrosis
Open accessAbstract
Cystic fibrosis is an autosomal recessive, monogenetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The gene defect was first described 25 years ago and much progress has been made since then in our understanding of how CFTR mutations cause disease and how this can be addressed therapeutically. CFTR is a transmembrane protein that transports ions across the surface of epithelial cells. CFTR dysfunction affects many organs; however, lung disease is responsible for the vast majority of morbidity and mortality in patients with cystic fibrosis. Prenatal diagnostics, newborn screening and new treatment algorithms are changing the incidence and the prevalence of the disease. Until recently, the standard of care in cystic fibrosis treatment focused on preventing and treating complications of the disease; now, novel treatment strategies directly targeting the ion channel abnormality are becoming available and it will be important to evaluate how these treatments affect disease progression and the quality of life of patients. In this Primer, we summarize the current knowledge, and provide an outlook on how cystic fibrosis clinical care and research will be affected by new knowledge and therapeutic options in the near future. For an illustrated summary of this Primer, visit: http://go.nature.com/4VrefN
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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
Identification of the Cystic Fibrosis Gene: Genetic Analysis
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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
Self-citationMolecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis
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Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
Van Goor, F., Hadida, S., Grootenhuis, P. D. J., Burton, B., Stack, J. H., Straley, K. S., Decker, C. J., Miller, M., McCartney, J., Olson, E. R., et al. (2011) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1105787108 · Cited by 1,088
Mucus clearance as a primary innate defense mechanism for mammalian airways
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Saiman, L., Marshall, B. C., Mayer-Hamblett, N., Burns, J. L., Quittner, A. L., Cibene, D. A., Coquillette, S., Fieberg, A. Y., Accurso, F. J., Campbell III, P. W., et al. (2003) · JAMA · doi:10.1001/jama.290.13.1749 · Cited by 958
Self-citationQuittner, A. L., Goldbeck, L., Abbott, J., Duff, A., Lambrecht, P., Solé, A., Tibosch, M. M., Bergsten Brucefors, A., Yüksel, H., Catastini, P., et al. (2014) · Thorax · doi:10.1136/thoraxjnl-2014-205983 · Cited by 524
Self-citationHigh treatment burden in adults with cystic fibrosis: challenges to disease self-management
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Cited by
Long‐term expanding human airway organoids for disease modeling
Sachs, N., Papaspyropoulos, A., Zomer‐van Ommen, D. D., Heo, I., Böttinger, L., Klay, D., Weeber, F., Huelsz‐Prince, G., Iakobachvili, N., Amatngalim, G. D., et al. (2019) · The EMBO Journal · doi:10.15252/embj.2018100300 · Cited by 1,082
Zhao, Z., Chen, X., Dowbaj, A. M., Sljukic, A., Bratlie, K., Lin, L., Fong, E. L. S., Balachander, G. M., Chen, Z., Soragni, A., et al. (2022) · Nature Reviews Methods Primers · doi:10.1038/s43586-022-00174-y · Cited by 1,082
Anion transport and supramolecular medicinal chemistry
Gale, P. A., Davis, J. T., & Quesada, R. (2017) · Chemical Society Reviews · doi:10.1039/c7cs00159b · Cited by 359
Zemanick, E. T., Taylor-Cousar, J. L., Davies, J., Gibson, R. L., Mall, M. A., McKone, E. F., McNally, P., Ramsey, B. W., Rayment, J. H., Rowe, S. M., et al. (2021) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.202102-0509oc · Cited by 305
Self-citationTaylor-Cousar, J. L., Mall, M. A., Ramsey, B. W., McKone, E. F., Tullis, E., Marigowda, G., McKee, C. M., Waltz, D., Moskowitz, S. M., Savage, J., et al. (2019) · ERJ Open Research · doi:10.1183/23120541.00082-2019 · Cited by 105
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Metadata
- Authors
- Ratjen, F., Bell, S. C., Rowe, S. M., Goss, C. H., Quittner, A. L., & Bush, A.
- Venue
- Nature Reviews Disease Primers
- Published
- 2015-05-14
- Volume
- 1
- Issue
- 1
- Pages
- 15010-15010
- ISSN
- 2056-676X (ISSN-L)
- OpenAlex
- W4211144794
- Cited by
- 546
- References
- 21
- FWCI
- 3.94
- Type
- journal article
- Language
- en
- Open access
- green
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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.
