Detalhe da pesquisa
1.
Enhanced delivery of peptide-morpholino oligonucleotides with a small molecule to correct splicing defects in the lung.
Nucleic Acids Res
; 49(11): 6100-6113, 2021 06 21.
Artigo
Inglês
| MEDLINE | ID: mdl-34107015
2.
Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration.
Eur Respir J
; 59(2)2022 02.
Artigo
Inglês
| MEDLINE | ID: mdl-34172469
3.
Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia.
Am J Respir Crit Care Med
; 203(10): 1275-1289, 2021 05 15.
Artigo
Inglês
| MEDLINE | ID: mdl-33321047
4.
Generation of renewable mouse intestinal epithelial cell monolayers and organoids for functional analyses.
BMC Cell Biol
; 19(1): 15, 2018 08 15.
Artigo
Inglês
| MEDLINE | ID: mdl-30111276
5.
Pharmacological Rescue of Conditionally Reprogrammed Cystic Fibrosis Bronchial Epithelial Cells.
Am J Respir Cell Mol Biol
; 56(5): 568-574, 2017 05.
Artigo
Inglês
| MEDLINE | ID: mdl-27983869
6.
Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770.
Am J Physiol Lung Cell Mol Physiol
; 311(3): L550-9, 2016 09 01.
Artigo
Inglês
| MEDLINE | ID: mdl-27402691
7.
Personalised medicine for non-classic cystic fibrosis resulting from rare CFTR mutations.
Eur Respir J
; 56(1)2020 07.
Artigo
Inglês
| MEDLINE | ID: mdl-32265312
8.
Cystic fibrosis airway inflammation enables elexacaftor/tezacaftor/ivacaftor-mediated rescue of N1303K CFTR mutation.
ERJ Open Res
; 10(1)2024 Jan.
Artigo
Inglês
| MEDLINE | ID: mdl-38226069
9.
The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model.
Eur Respir J
; 52(6)2018 12.
Artigo
Inglês
| MEDLINE | ID: mdl-30287473
10.
A Novel Co-Culture Model Reveals Enhanced CFTR Rescue in Primary Cystic Fibrosis Airway Epithelial Cultures with Persistent Pseudomonas aeruginosa Infection.
Cells
; 12(22)2023 11 13.
Artigo
Inglês
| MEDLINE | ID: mdl-37998353
11.
A small-molecule inhibitor and degrader of the RNF5 ubiquitin ligase.
Mol Biol Cell
; 33(13): ar120, 2022 11 01.
Artigo
Inglês
| MEDLINE | ID: mdl-36074076
12.
A PI3Kγ mimetic peptide triggers CFTR gating, bronchodilation, and reduced inflammation in obstructive airway diseases.
Sci Transl Med
; 14(638): eabl6328, 2022 03 30.
Artigo
Inglês
| MEDLINE | ID: mdl-35353541
13.
DNAJB12 and Hsp70 triage arrested intermediates of N1303K-CFTR for endoplasmic reticulum-associated autophagy.
Mol Biol Cell
; 32(7): 538-553, 2021 04 01.
Artigo
Inglês
| MEDLINE | ID: mdl-33534640
14.
Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.
Front Pharmacol
; 12: 628722, 2021.
Artigo
Inglês
| MEDLINE | ID: mdl-33859562
15.
Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment.
J Cyst Fibros
; 19(5): 746-751, 2020 09.
Artigo
Inglês
| MEDLINE | ID: mdl-32536510
16.
Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.
JCI Insight
; 3(3)2018 02 08.
Artigo
Inglês
| MEDLINE | ID: mdl-29415893
17.
Nasospheroids permit measurements of CFTR-dependent fluid transport.
JCI Insight
; 2(22)2017 11 16.
Artigo
Inglês
| MEDLINE | ID: mdl-29202459
18.
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.
Sci Transl Med
; 6(246): 246ra96, 2014 Jul 23.
Artigo
Inglês
| MEDLINE | ID: mdl-25101886
19.
Thymosin α-1 does not correct F508del-CFTR in cystic fibrosis airway epithelia.
JCI Insight
; 4(7)2019 04 04.
Artigo
Inglês
| MEDLINE | ID: mdl-30944255