The DOI matches its Crossref record; 32 of the 32 references it cites were found in a registry or an index.
Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis
Open accessAbstract
A breathtaking tale of sticky mucus: Patients with cystic fibrosis have difficulty breathing because their airways are clogged with thick mucus. Does this mucus accumulate because there is a defect in the way it is produced? Or does it accumulate because of other disease features, such as dehydration or airway wall remodeling? Distinguishing between these possibilities is important for future drug development. In a study of piglets with cystic fibrosis, Hoegger et al. identify mucus production as the primary defect (see the Perspective by Wine). The airway glands of the piglets synthesized strands of mucus normally, but the strands were never released and stayed tethered to the gland ducts. Science , this issue p. 818 ; see also p. 730
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Cites
Update on Key Emerging Challenges in Cystic Fibrosis
Jennings, M. T., Riekert, K. A., & Boyle, M. P. (2014) · Medical Principles and Practice · doi:10.1159/000357646 · Cited by 3,648
Airway Mucus Function and Dysfunction
Fahy, J. V., & Dickey, B. F. (2010) · New England Journal of Medicine · doi:10.1056/nejmra0910061 · Cited by 1,722
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Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
Pezzulo, A. A., Tang, X. X., Hoegger, M. J., Abou Alaiwa, M. H., Ramachandran, S., Moninger, T. O., Karp, P. H., Wohlford-Lenane, C. L., Haagsman, H. P., van Eijk, M., et al. (2012) · Nature · doi:10.1038/nature11130 · Cited by 808
Self-citationDisruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs
Rogers, C. S., Stoltz, D. A., Meyerholz, D. K., Ostedgaard, L. S., Rokhlina, T., Taft, P. J., Rogan, M. P., Pezzulo, A. A., Karp, P. H., Itani, O. A., et al. (2008) · Science · doi:10.1126/science.1163600 · Cited by 749
Self-citationMucociliary clearance in the airways.
Wanner, A., Salathé, M., & O'Riordan, T. G. (1996) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/ajrccm.154.6.8970383 · Cited by 731
Submucosal glands are the predominant site of CFTR expression in the human bronchus
Engelhardt, J. F., Yankaskas, J. R., Ernst, S. A., Yang, Y., Marino, C. R., Boucher, R. C., Cohn, J. A., & Wilson, J. M. (1992) · Nature Genetics · doi:10.1038/ng1192-240 · Cited by 653
Cystic Fibrosis Pigs Develop Lung Disease and Exhibit Defective Bacterial Eradication at Birth
Stoltz, D. A., Meyerholz, D. K., Pezzulo, A. A., Ramachandran, S., Rogan, M. P., Davis, G. J., Hanfland, R. A., Wohlford-Lenane, C., Dohrn, C. L., Bartlett, J. A., et al. (2010) · Science Translational Medicine · doi:10.1126/scitranslmed.3000928 · Cited by 495
Self-citationPoulsen, J. H., Fischer, H., Illek, B., & Machen, T. E. (1994) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.91.12.5340 · Cited by 420
Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis
Quinton, P. M. (2008) · The Lancet · doi:10.1016/s0140-6736(08)61162-9 · Cited by 381
Cited by
Elborn, J. S. (2016) · The Lancet · doi:10.1016/s0140-6736(16)00576-6 · Cited by 1,805
A revised airway epithelial hierarchy includes CFTR-expressing ionocytes
Montoro, D. T., Haber, A. L., Biton, M., Vinarsky, V., Lin, B., Birket, S. E., Yuan, F., Chen, S., Leung, H. M., Villoria, J., et al. (2018) · Nature · doi:10.1038/s41586-018-0393-7 · Cited by 1,206
Importance of the pig as a human biomedical model
Lunney, J. K., Van Goor, A., Walker, K. E., Hailstock, T., Franklin, J., & Dai, C. (2021) · Science Translational Medicine · doi:10.1126/scitranslmed.abd5758 · Cited by 801
Role of CFTR in epithelial physiology
Saint-Criq, V., & Gray, M. A. (2017) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-016-2391-y · Cited by 433
Pulmonary neuroendocrine cells amplify allergic asthma responses
Sui, P., Wiesner, D. L., Xu, J., Zhang, Y., Lee, J., Van Dyken, S., Lashua, A., Yu, C., Klein, B. S., Locksley, R. M., et al. (2018) · Science · doi:10.1126/science.aan8546 · Cited by 428
Metadata
- Authors
- Hoegger, M. J., Fischer, A. J., McMenimen, J. D., Ostedgaard, L. S., Tucker, A. J., Awadalla, M. A., Moninger, T. O., Michalski, A. S., Hoffman, E. A., Zabner, J., Stoltz, D. A., & Welsh, M. J.
- Venue
- Science
- Published
- 2014-08-15
- Volume
- 345
- Issue
- 6198
- Pages
- 818-822
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2044380511
- Cited by
- 368
- References
- 32
- FWCI
- 32.23
- Type
- journal article
- Language
- en
- Open access
- green
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- CoChat. (2026). Citation check: Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis. https://app.cochat.ai/papers/10.1126/science.1255825
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- Hoegger, M. J., Fischer, A. J., McMenimen, J. D., Ostedgaard, L. S., Tucker, A. J., Awadalla, M. A., Moninger, T. O., Michalski, A. S., Hoffman, E. A., Zabner, J., Stoltz, D. A., & Welsh, M. J. (2014). Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis. Science. https://doi.org/10.1126/science.1255825
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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.
