Resveratrol and ivacaftor are additive G551D CFTR-channel potentiators: therapeutic implications for cystic fibrosis sinus disease.
Aminophenols
/ pharmacology
Animals
Cells, Cultured
Chlorides
/ metabolism
Cystic Fibrosis
/ drug therapy
Cystic Fibrosis Transmembrane Conductance Regulator
/ genetics
Drug Synergism
Drug Therapy, Combination
Humans
Ion Transport
/ drug effects
Mucociliary Clearance
/ drug effects
Mutation
/ genetics
Nasal Mucosa
/ pathology
Paranasal Sinus Diseases
/ drug therapy
Quinolones
/ pharmacology
Rats
Resveratrol
/ pharmacology
Thyroid Gland
/ drug effects
CFTR
CFTR potentiator
G551D
chronic rhinosinusitis
chronic sinusitis
cystic fibrosis
ivacaftor
mucociliary clearance
resveratrol
sinusitis
Journal
International forum of allergy & rhinology
ISSN: 2042-6984
Titre abrégé: Int Forum Allergy Rhinol
Pays: United States
ID NLM: 101550261
Informations de publication
Date de publication:
01 2019
01 2019
Historique:
received:
03
03
2018
revised:
19
06
2018
accepted:
15
07
2018
pubmed:
29
8
2018
medline:
18
12
2019
entrez:
29
8
2018
Statut:
ppublish
Résumé
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in defective Cl Fisher rat thyroid cells (FRT) transfected with G551D CFTR and human sinonasal epithelial cells (HSNE) containing the CFTR G551D mutation were subjected to pharmacologic manipulation of transepithelial ion transport in Ussing chambers. Activity was further evaluated using whole-cell patch clamp methods in G551D FRT cells. In G551D FRT cells, resveratrol (100 μM) and ivacaftor (10 μM) significantly increased Cl Additive improvement in G551D CFTR-mediated Cl
Sections du résumé
BACKGROUND
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in defective Cl
METHODS
Fisher rat thyroid cells (FRT) transfected with G551D CFTR and human sinonasal epithelial cells (HSNE) containing the CFTR G551D mutation were subjected to pharmacologic manipulation of transepithelial ion transport in Ussing chambers. Activity was further evaluated using whole-cell patch clamp methods in G551D FRT cells.
RESULTS
In G551D FRT cells, resveratrol (100 μM) and ivacaftor (10 μM) significantly increased Cl
CONCLUSION
Additive improvement in G551D CFTR-mediated Cl
Identifiants
pubmed: 30152192
doi: 10.1002/alr.22202
pmc: PMC6318032
mid: NIHMS984861
doi:
Substances chimiques
Aminophenols
0
CFTR protein, human
0
Chlorides
0
Quinolones
0
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
ivacaftor
1Y740ILL1Z
Resveratrol
Q369O8926L
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
100-105Subventions
Organisme : NIDDK NIH HHS
ID : P30 DK072482
Pays : United States
Organisme : NHLBI NIH HHS
ID : R35 HL135816
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL133006
Pays : United States
Informations de copyright
© 2018 ARS-AAOA, LLC.
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