4.5 Article

Airway Epithelial Cultures of Children with Esophageal Atresia as a Model to Study Respiratory Tract Disorders

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CHILDREN-BASEL
卷 10, 期 6, 页码 -

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MDPI
DOI: 10.3390/children10061020

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esophageal atresia; tracheal anomaly; airway epithelium; organoids; primary ciliary dyskinesia

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Esophageal atresia (EA) is a rare birth defect with respiratory disorders as a major cause of morbidity. This study aimed to evaluate the feasibility of culturing and characterizing motile cilia function in airway epithelial cell cultures and 3D organoids derived from EA patients. The results showed that EA patient-derived airway epithelial cultures and organoids displayed normal motile cilia function, suggesting their potential use in studying the role of epithelial function in respiratory disorders in EA.
Esophageal atresia (EA) is a rare birth defect in which respiratory tract disorders are a major cause of morbidity. It remains unclear whether respiratory tract disorders are in part caused by alterations in airway epithelial cell functions such as the activity of motile cilia. This can be studied using airway epithelial cell culture models of patients with EA. Therefore, the aim of this study was to evaluate the feasibility to culture and functionally characterize motile cilia function in the differentiated air-liquid interface cultured airway epithelial cells and 3D organoids derived from nasal brushings and bronchoalveolar lavage (BAL) fluid from children with EA. We demonstrate the feasibility of culturing differentiated airway epithelia and organoids of nasal brushings and BAL fluid of children with EA, which display normal motile cilia function. EA patient-derived airway epithelial cultures can be further used to examine whether alterations in epithelial functions contribute to respiratory disorders in EA.

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