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In cystic fibrosis (CF), loss of CF transmembrane conductance regulator (CFTR)-dependent bicarbonate secretion precipitates the accumulation of viscous mucus in the lumen of respiratory and gastrointestinal epithelial tissues. We investigated whether the combination of elexacaftor (ELX), ivacaftor (...
ORGANISM(S): Homo sapiens 
2021-12-14 | GSE182347 | GEO
Rescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers
Genomics
Sputum microbiota in people with cystic fibrosis starting on Elexacaftor/Tezacaftor/Ivacaftor
Rationale: We recently demonstrated that the triple combination CFTR modulator therapy elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) improves lung ventilation and airway mucus plugging determined by multiple-breath washout and magnetic resonance imaging in CF patients with at least one F508del alle...
ORGANISM(S): Homo sapiens (Human) 
2023-06-22 | PXD041844 | Pride
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