ArticleAmerican journal of physiology. Lung cellular and molecular physiology2014
Deletion of airway cilia results in noninflammatory bronchiectasis and hyperreactive airways.
Article in American journal of physiology. Lung cellular and molecular physiology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 38 citations in OpenAlex.
- Airway mucus plugs in asthma and chronic obstructive pulmonary disease: pathobiology, imaging, and implications for clinical trials.American journal of respiratory and critical care medicine · 2026Review
- Primary and motile cilia in the efferent ductules: a role for IFT88 in maintaining male fertility.Cellular and molecular life sciences : CMLS · 2026Article
- ARMC2 loss impairs cilia structure and leads to primary ciliary dyskinesia symptoms in mouse organs.Frontiers in cell and developmental biology · 2026Article
- Inducible Ift88-deficient mice show features consistent with mild pulmonary hypertension.Physiological reports · 2026Article
- Role of intraflagellar transport protein IFT140 in the formation and function of motile cilia in mammals.Cellular and molecular life sciences : CMLS · 2025Article
- Ciliary and Non-Ciliary Roles of IFT88 in Development and Diseases.International journal of molecular sciences · 2025Review
- T cell-expressed Ift88 is required for proper thymocyte differentiation in mice.Physiological reports · 2024Article
- Formation and function of multiciliated cells.The Journal of cell biology · 2024Review
- Airway ciliated cells in adult lung homeostasis and COPD.European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Mechanical stretch promotes apoptosis and impedes ciliogenesis of primary human airway basal stem cells.Respiratory research · 2023Article
- Down-expression of Foxj1 on airway epithelium with impaired cilia architecture in non-cystic fibrosis bronchiectasis implies disease severity.The clinical respiratory journal · 2023Article
- Mice with lung airway ciliopathy develop persistentFrontiers in immunology · 2022Article
- Novel non-cystic features of polycystic kidney disease: having new eyes or seeking new landscapes.Clinical kidney journal · 2021Review
- Motile cilia genetics and cell biology: big results from little mice.Cellular and molecular life sciences : CMLS · 2021Review
- Characterizing smoking-induced transcriptional heterogeneity in the human bronchial epithelium at single-cell resolution.Science advances · 2019Article
- TRPPing up bronchiectasis.American journal of physiology. Lung cellular and molecular physiology · 2019Article
- Mucociliary Clearance in Mice Measured by Tracking Trans-tracheal Fluorescence of Nasally Aerosolized Beads.Scientific reports · 2018Article
- Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneity.Lancet (London, England) · 2018Review
- Motile Ciliary Disorders in Chronic Airway Inflammatory Diseases: Critical Target for Interventions.Current allergy and asthma reports · 2018Review
- Aberrant epithelial remodeling with impairment of cilia architecture in non-cystic fibrosis bronchiectasis.Journal of thoracic disease · 2018Article
Corrections and comments
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Authors and funding
18 authors at 4 institutions in 1 country.
Funding
Abstract
The mechanisms for the development of bronchiectasis and airway hyperreactivity have not been fully elucidated. Although genetic, acquired diseases and environmental influences may play a role, it is also possible that motile cilia can influence this disease process. We hypothesized that deletion of a key intraflagellar transport molecule, IFT88, in mature mice causes loss of cilia, resulting in airway remodeling. Airway cilia were deleted by knockout of IFT88, and airway remodeling and pulmonary function were evaluated. In IFT88(-) mice there was a substantial loss of airway cilia on respiratory epithelium. Three months after the deletion of cilia, there was clear evidence for bronchial remodeling that was not associated with inflammation or apparent defects in mucus clearance. There was evidence for airway epithelial cell hypertrophy and hyperplasia. IFT88(-) mice exhibited increased airway reactivity to a methacholine challenge and decreased ciliary beat frequency in the few remaining cells that possessed cilia. With deletion of respiratory cilia there was a marked increase in the number of club cells as seen by scanning electron microscopy. We suggest that airway remodeling may be exacerbated by the presence of club cells, since these cells are involved in airway repair. Club cells may be prevented from differentiating into respiratory epithelial cells because of a lack of IFT88 protein that is necessary to form a single nonmotile cilium. This monocilium is a prerequisite for these progenitor cells to transition into respiratory epithelial cells. In conclusion, motile cilia may play an important role in controlling airway structure and function.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.