4.5 Article

Lipid-driven CFTR clustering is impaired in cystic fibrosis and restored by corrector drugs

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CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells

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Summary: In cystic fibrosis, a deficiency in CFTR function leads to chronic lung disease. Abnormalities in lipid metabolism and oxidative stress were observed in CF airway epithelium, indicating potential roles in CF lung pathology progression. Treatment with antioxidants or CFTR modulators may require a combination approach for correction of the CF phenotype.

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