Detalhe da pesquisa
1.
Requirements for efficient correction of ΔF508 CFTR revealed by analyses of evolved sequences.
Cell
; 148(1-2): 164-74, 2012 Jan 20.
Artigo
Inglês
| MEDLINE | ID: mdl-22265409
2.
Open reading frame correction using splice-switching antisense oligonucleotides for the treatment of cystic fibrosis.
Proc Natl Acad Sci U S A
; 119(3)2022 01 18.
Artigo
Inglês
| MEDLINE | ID: mdl-35017302
3.
KCa3.1 potentiation stimulates Cl- secretion in F508del and G551D CFTR-corrected primary human bronchial epithelial cells.
Am J Physiol Cell Physiol
; 323(4): C1215-C1230, 2022 Oct 01.
Artigo
Inglês
| MEDLINE | ID: mdl-36062876
4.
Antisense oligonucleotide-mediated correction of CFTR splicing improves chloride secretion in cystic fibrosis patient-derived bronchial epithelial cells.
Nucleic Acids Res
; 48(13): 7454-7467, 2020 07 27.
Artigo
Inglês
| MEDLINE | ID: mdl-32520327
5.
Identification of the amino acids inserted during suppression of CFTR nonsense mutations and determination of their functional consequences.
Hum Mol Genet
; 26(16): 3116-3129, 2017 08 15.
Artigo
Inglês
| MEDLINE | ID: mdl-28575328
6.
Restoration of CFTR Activity in Ducts Rescues Acinar Cell Function and Reduces Inflammation in Pancreatic and Salivary Glands of Mice.
Gastroenterology
; 153(4): 1148-1159, 2017 10.
Artigo
Inglês
| MEDLINE | ID: mdl-28634110
7.
Cystic Fibrosis, Cystic Fibrosis Transmembrane Conductance Regulator and Drugs: Insights from Cellular Trafficking.
Handb Exp Pharmacol
; 245: 385-425, 2018.
Artigo
Inglês
| MEDLINE | ID: mdl-29460152
8.
Evidence against resveratrol as a viable therapy for the rescue of defective ΔF508 CFTR.
Biochim Biophys Acta
; 1850(11): 2377-84, 2015 Nov.
Artigo
Inglês
| MEDLINE | ID: mdl-26342647
9.
In vitro modulator responsiveness of 655 CFTR variants found in people with cystic fibrosis.
J Cyst Fibros
; 2024 Feb 21.
Artigo
Inglês
| MEDLINE | ID: mdl-38388235
10.
Effect of apical hyperosmotic sodium challenge and amiloride on sodium transport in human bronchial epithelial cells from cystic fibrosis donors.
Am J Physiol Cell Physiol
; 305(11): C1114-22, 2013 Dec 01.
Artigo
Inglês
| MEDLINE | ID: mdl-23986197
11.
Translating in vitro CFTR rescue into small molecule correctors for cystic fibrosis using the Library of Integrated Network-based Cellular Signatures drug discovery platform.
CPT Pharmacometrics Syst Pharmacol
; 11(2): 240-251, 2022 02.
Artigo
Inglês
| MEDLINE | ID: mdl-34877817
12.
CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor.
JCI Insight
; 7(6)2022 03 22.
Artigo
Inglês
| MEDLINE | ID: mdl-35315358
13.
Sphingomyelinase decreases transepithelial anion secretion in airway epithelial cells in part by inhibiting CFTR-mediated apical conductance.
Physiol Rep
; 9(15): e14928, 2021 08.
Artigo
Inglês
| MEDLINE | ID: mdl-34382377
14.
Mechanistic analysis and significance of sphingomyelinase-mediated decreases in transepithelial CFTR currents in nHBEs.
Physiol Rep
; 9(17): e15023, 2021 09.
Artigo
Inglês
| MEDLINE | ID: mdl-34514718
15.
Integrative chemogenomic analysis identifies small molecules that partially rescue ΔF508-CFTR for cystic fibrosis.
CPT Pharmacometrics Syst Pharmacol
; 10(5): 500-510, 2021 05.
Artigo
Inglês
| MEDLINE | ID: mdl-33934548
16.
Small molecule correctors of F508del-CFTR discovered by structure-based virtual screening.
J Comput Aided Mol Des
; 24(12): 971-91, 2010 Dec.
Artigo
Inglês
| MEDLINE | ID: mdl-20976528
17.
Dynasore inhibits removal of wild-type and DeltaF508 cystic fibrosis transmembrane conductance regulator (CFTR) from the plasma membrane.
Biochem J
; 421(3): 377-85, 2009 Jul 15.
Artigo
Inglês
| MEDLINE | ID: mdl-19442237
18.
CFTR modulator theratyping: Current status, gaps and future directions.
J Cyst Fibros
; 18(1): 22-34, 2019 01.
Artigo
Inglês
| MEDLINE | ID: mdl-29934203
19.
A segment of gamma ENaC mediates elastase activation of Na+ transport.
J Gen Physiol
; 130(6): 611-29, 2007 Dec.
Artigo
Inglês
| MEDLINE | ID: mdl-17998393
20.
Identification and membrane localization of electrogenic sodium bicarbonate cotransporters in Calu-3 cells.
Biochim Biophys Acta
; 1762(7): 704-10, 2006 Jul.
Artigo
Inglês
| MEDLINE | ID: mdl-16857349