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Article

Anti-Infectives Restore ORKAMBI® Rescue of F508del-CFTR Function in Human Bronchial Epithelial Cells Infected with Clinical Strains of P. aeruginosa.

Biomolecules - 19 Feb 2020

Laselva Onofrio, Stone Tracy A, Bear Christine E, Deber Charles M

Abstract excerpt

Chronic infection and inflammation are the primary causes of declining lung function in Cystic Fibrosis (CF) patients. ORKAMBI® (Lumacaftor-Ivacaftor) is an approved combination therapy for Cystic Fibrosis (CF) patients bearing the most common mutation, F508del, in the cystic fibrosis conductance regulator (CFTR) protein. It has been previously shown that ORKAMBI®-mediated rescue of CFTR is reduced by a...

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PMID: 32092967
DOI: 10.3390/biom10020334
PMCID: PMC7072183

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