2017
DOI: 10.1038/s41467-017-00813-z
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MicroRNA-9 downregulates the ANO1 chloride channel and contributes to cystic fibrosis lung pathology

Abstract: Cystic fibrosis results from reduced cystic fibrosis transmembrane conductance regulator protein activity leading to defective epithelial ion transport. Ca2+-activated Cl− channels mediate physiological functions independently of cystic fibrosis transmembrane conductance regulator. Anoctamin 1 (ANO1/TMEM16A) was identified as the major Ca2+-activated Cl− channel in airway epithelial cells, and we recently demonstrated that downregulation of the anoctamin 1 channel in cystic fibrosis patients contributes to dis… Show more

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Cited by 63 publications
(59 citation statements)
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“…The calcium-activated chloride ion channel, ANO1, has also been shown to contribute to CF pathology and is downregulated in CF epithelium [126]. ANO1 is targeted by miR-9, which is increased in fully differentiated bronchial epithelial cells from CF patients [127].…”
Section: Mirnas In Cfmentioning
confidence: 99%
“…The calcium-activated chloride ion channel, ANO1, has also been shown to contribute to CF pathology and is downregulated in CF epithelium [126]. ANO1 is targeted by miR-9, which is increased in fully differentiated bronchial epithelial cells from CF patients [127].…”
Section: Mirnas In Cfmentioning
confidence: 99%
“…For viral infections, MucilAir™-Pool airway epithelia were reconstituted with a mixture of human nasal cells isolated from 14 different donors, and cultured at the air-liquid interface (ALI) in MucilAir™ culture medium (EP04MM), ready-to-use, chemically defined, serum-free (Epithelix Sàrl, Geneva, Switzerland), in 24-well plates with 6.5-mm Transwell ® inserts (cat #3470, Corning Incorporated, Tweksbury, USA). The MucilAir™ models are stable for months and generate reproducible data in several domains, such as toxicology, therapeutic drug study or disease model (Baxter et al, 2015), (Balogh Sivars et al, 2017), (Rocca et al, 2016), (Sonneville et al, 2017).…”
Section: Mucilair™ Culturingmentioning
confidence: 99%
“…They improve mucociliary clearance, decrease inflammation or fight infection 2 . One of the new therapeutic strategies proposed in the literature is based on the stimulation of an alternative pathway to that involving CFTR for the transfer of chloride ions, such as that induced by the chloride channel ANO1 (Anoctamin-1, also called TMEM16A [transmembrane protein 16A]) ( Caputo et al, 2008 ; Schroeder et al, 2008 ; Yang et al, 2008 ; Sonneville et al, 2017 ), which could also participate in the activation of CFTR in the cell membrane ( Benedetto et al, 2017 ). Another proposed emerging approach is based on modulating the expression of the microRNAs that regulate target RNAs such as CFTR.…”
Section: Cystic Fibrosis and Therapeutic Approachesmentioning
confidence: 99%