Acquired CFTR dysfunction and dense distribution of ionocytes in nasal mucosa of children with CRS
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Published on: July 4, 2018
Yang Han1, Chao Jia2, Tieshan Wang3
1Department of Otolaryngology, Head and Neck Surgery, National Center for Children's Health, Beijing Children's Hospital, Capital Medical University, 56 NanLishi Road, Xicheng District, Beijing, 100045, China.
Insights
Children with chronic rhinosinusitis (CRS) have fewer ionocytes and reduced cystic fibrosis transmembrane conductance regulator (CFTR) function in their nasal lining. Inflammation impacts ionocyte distribution and CFTR expression, potentially linking to polyp development.
Area of Science:
- Respiratory Medicine
- Cell Biology
- Pediatric Otolaryngology
Background:
- Ionocytes are specialized airway epithelial cells with high expression of the cystic fibrosis transmembrane conductance regulator (CFTR).
- Understanding ionocyte and CFTR roles is crucial for airway diseases.
Purpose of the Study:
- To investigate ionocyte morphology, distribution, and CFTR function in pediatric nasal mucosa.
- To examine these factors in children with chronic rhinosinusitis with nasal polyps (CRSwNP).
Main Methods:
- Flow cytometry analyzed ionocyte numbers and CFTR function in exfoliated nasal cells from 101 children.
- RNAscope identified FOXI1 and CFTR expression in nasal mucosa and polyp tissues from 10 CRSwNP patients.
Main Results:
- CRS patients exhibited fewer ionocytes and decreased CFTR function.
- CFTR expression in CRS nasal mucosa was focally dense and increased with inflammation.
- Ionocytes in nasal polyps often lacked CFTR expression and typical morphology.
Conclusions:
- Reduced ionocyte numbers and impaired CFTR function are observed in pediatric CRS.
- Inflammation influences ionocyte and CFTR distribution.
- Loss of CFTR expression and altered ionocyte shape may contribute to nasal polyp formation.
Background:
Ionocytes are rare cells in airway epithelium characterized by a high expression of CFTR.
Objectives:
To investigate the morphology and distribution of ionocytes and the function of CFTR in the nasal mucosal epithelium of children.
Methods:
The exfoliated cells of nasal mucosa from 101 children were detected using flow cytometry to analyze the number of ionocytes and CFTR and the difference of CFTR function. Nasal mucosa and polyps were collected from 10 children with CRSwNP. The RNAscope of FOXI1 and CFTR was detected in pathological paraffin sections. The expression and distribution of ionocytes and CFTR in nasal mucosa and polyp epithelium were observed.
Results:
In CRS patients, the number of ionocytes in the nasal epithelium was lower and the number of ionocytes that did not express CFTR was higher, and the function of CFTR was also decreased. The expression of CFTR in the nasal mucosa of CRS showed the characteristics of local dense distribution and increased as the inflammation expanded. The ionocytes were "tadpole-shaped" in the epithelium and gathered in the area of high CFTR expression, the intracellular CFTR was expanded in clusters. Ionocytes that did not express CFTR was more common in the nasal polyps.
Conclusions:
The number of ionocytes and the function of CFTR in nasal mucosa of CRS patients decreased. With the expansion of inflammation, CFTR and ionocytes showed more obvious dense distribution. Some ionocytes lost the expression of CFTR and did not show the "tadpole" shape, which may be related to the occurrence of polyps.
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