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Submucosal gland dysfunction as a primary defect in cystic fibrosis
Danieli Salinas1, Peter M Haggie, Jay R Thiagarajah
1Department of Medicine, University of California, San Francisco, California 94143-0521, USA.
Summary
Defective submucosal gland function, characterized by increased viscosity and reduced secretion rate, is an early defect in cystic fibrosis (CF) airways. These findings suggest early therapies targeting gland function may mitigate CF lung disease.
Area of Science:
- Respiratory Medicine
- Cell Biology
- Genetics
Background:
- Submucosal gland dysfunction is implicated in cystic fibrosis (CF) airway disease.
- Early functional defects in CF submucosal glands are not well characterized.
Purpose of the Study:
- To investigate submucosal gland function in CF subjects with minimal clinical disease.
- To determine if defective gland function is an early primary defect in CF.
Main Methods:
- Functional assays on nasal biopsies from CF and non-CF subjects (ages 2-22).
- Measurement of gland fluid secretion rate and viscosity using microscopy and fluorescence recovery after photobleaching.
- Quantitative histology to assess gland size and morphology.
Main Results:
- CF submucosal gland fluid viscosity was significantly higher (4.9-fold) than in non-CF controls (2.2-fold).
- Gland fluid secretion rate was reduced 2.7-fold in CF specimens compared to controls.
- Histology showed similar submucosal gland size and morphology between CF and non-CF groups.
Conclusions:
- Defective submucosal gland function, including increased viscosity and reduced secretion, is an early, primary defect in CF.
- Therapeutic strategies aimed at normalizing CF submucosal gland function early may prevent or reduce CF lung disease progression.