Correction of CFTR function in nasal epithelial cells from cystic fibrosis patients predicts improvement of respiratory function by CFTR modulators

Scientific Reports

Volumn 7, Issue 1, 2017, Pages

  Pranke, Iwona M ^a   Hatton, Aurélie ^a   Simonin, Juliette ^a   Jais, Jean Philippe ^b   Le Pimpec Barthes, Françoise ^c   Carsin, Ania ^d   Bonnette, Pierre ^e   Fayon, Michael ^f   Stremler Le Bel, Nathalie ^d   Grenet, Dominique ^e   Thumerel, Matthieu ^f   Mazenq, Julie ^d   Urbach, Valerie ^a   Mesbahi, Myriam ^a   Girodon Boulandet, Emanuelle ^g   Hinzpeter, Alexandre ^a   Edelman, Aleksander ^a   Sermet Gaudelus, Isabelle ^a,b  

^a INSTITUT NECKER ENFANTS MALADES   (France)

^b HÔPITAL NECKER ENFANTS MALADES   (France)

^c HÔPITAL EUROPÉEN GEORGES POMPIDOU   (France)

^d Service de Pneumo Pédiatrie   (France)

^e FOCH HOSPITAL   (France)

^f HÔPITAL PELLEGRIN   (France)

^g NONE   (France)

Author keywords

[No Author keywords available]

Indexed keywords

1,3 BENZODIOXOLE DERIVATIVE; AMINOPYRIDINE DERIVATIVE; BIOLOGICAL MARKER; CFTR PROTEIN, HUMAN; CHLORIDE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; INDOLE DERIVATIVE; LUMACAFTOR; TEZACAFTOR;

CELL CULTURE; CELL CULTURE TECHNIQUE; CYSTIC FIBROSIS; EPITHELIUM CELL; GENETICS; HOMOZYGOTE; HUMAN; LUNG FUNCTION TEST; METABOLISM; MUTATION; NOSE MUCOSA; PATHOPHYSIOLOGY; TREATMENT OUTCOME;

AMINOPYRIDINES; BENZODIOXOLES; BIOMARKERS; CELL CULTURE TECHNIQUES; CELLS, CULTURED; CHLORIDES; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; EPITHELIAL CELLS; HOMOZYGOTE; HUMANS; INDOLES; MUTATION; NASAL MUCOSA; RESPIRATORY FUNCTION TESTS; TREATMENT OUTCOME;

EID: 85027236424     PISSN: None     EISSN: 20452322     Source Type: Journal    
DOI: 10.1038/s41598-017-07504-1     Document Type: Article

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