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Article Dans Une Revue Journal of Cystic Fibrosis Année : 2022

Risk factors for Pseudomonas aeruginosa airway infection and lung function decline in children with cystic fibrosis

Résumé

Cystic fibrosis (CF) is the most common of rare hereditary diseases in Caucasians, and it is estimated to affect 75,000 patients globally. CF is a complex disease due to the multiplicity of mutations found in the CF transmembrane conductance regulator (CFTR) gene causing the CFTR protein to become dysfunctional. Correctors and potentiators have demonstrated good clinical outcomes for patients with specific gene mutations; however, there are still patients for whom those treatments are not suitable and require alternative CFTR-independent strategies. Although CFTR is the main chloride channel in the lungs, others could, e.g., anoctamin-1 (ANO1 or TMEM16A), compensate for the deficiency of CFTR. This review summarizes the current knowledge on calcium-activated chloride channel (CaCC) ANO1 and presents ANO1 as an exciting target in CF.
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hal-03827738 , version 1 (22-07-2024)

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Julie Mésinèle, Manon Ruffin, Astrid Kemgang, Loïc Guillot, Pierre-Yves Boëlle, et al.. Risk factors for Pseudomonas aeruginosa airway infection and lung function decline in children with cystic fibrosis. Journal of Cystic Fibrosis, 2022, 21 (1), pp.45-51. ⟨10.1016/j.jcf.2021.09.017⟩. ⟨hal-03827738⟩
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