Detalhe da pesquisa
1.
Rescue of multiple class II CFTR mutations by elexacaftor+tezacaftor+ivacaftor mediated in part by the dual activities of elexacaftor as both corrector and potentiator.
Eur Respir J
; 57(6)2021 06.
Artigo
em Inglês
| MEDLINE | ID: mdl-33303536
2.
Attenuation of Phosphorylation-dependent Activation of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) by Disease-causing Mutations at the Transmission Interface.
J Biol Chem
; 292(5): 1988-1999, 2017 02 03.
Artigo
em Inglês
| MEDLINE | ID: mdl-28003367
3.
Structural basis for alginate secretion across the bacterial outer membrane.
Proc Natl Acad Sci U S A
; 108(32): 13083-8, 2011 Aug 09.
Artigo
em Inglês
| MEDLINE | ID: mdl-21778407
4.
Comparison of a novel potentiator of CFTR channel activity to ivacaftor in ameliorating mucostasis caused by cigarette smoke in primary human bronchial airway epithelial cells.
bioRxiv
; 2024 Mar 04.
Artigo
em Inglês
| MEDLINE | ID: mdl-38496440
5.
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner.
J Biol Chem
; 287(44): 36639-49, 2012 Oct 26.
Artigo
em Inglês
| MEDLINE | ID: mdl-22942289
6.
Correlation of Electrophysiological and Fluorescence-Based Measurements of Modulator Efficacy in Nasal Epithelial Cultures Derived from People with Cystic Fibrosis.
Cells
; 12(8)2023 04 17.
Artigo
em Inglês
| MEDLINE | ID: mdl-37190083
7.
Cost-effectiveness analysis of genetic tools to predict treatment response in patients with cystic fibrosis.
J Cyst Fibros
; 22(5): 933-940, 2023 Sep.
Artigo
em Inglês
| MEDLINE | ID: mdl-37100704
8.
Clinical and functional efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis carrying the N1303K mutation.
J Cyst Fibros
; 22(6): 1062-1069, 2023 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-37331863
9.
Targeting the regulation of CFTR channels.
Biochem J
; 435(2): e1-4, 2011 Apr 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-21726198
10.
The reconstituted Escherichia coli MsbA protein displays lipid flippase activity.
Biochem J
; 429(1): 195-203, 2010 Jul 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-20412049
11.
A new platform for high-throughput therapy testing on iPSC-derived lung progenitor cells from cystic fibrosis patients.
Stem Cell Reports
; 16(11): 2825-2837, 2021 11 09.
Artigo
em Inglês
| MEDLINE | ID: mdl-34678210
12.
Preclinical Studies of a Rare CF-Causing Mutation in the Second Nucleotide Binding Domain (c.3700A>G) Show Robust Functional Rescue in Primary Nasal Cultures by Novel CFTR Modulators.
J Pers Med
; 10(4)2020 Nov 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-33167369
13.
The CF Canada-Sick Kids Program in individual CF therapy: A resource for the advancement of personalized medicine in CF.
J Cyst Fibros
; 18(1): 35-43, 2019 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-29685812
14.
Interaction of the P-glycoprotein multidrug efflux pump with cholesterol: effects on ATPase activity, drug binding and transport.
Biochemistry
; 47(51): 13686-98, 2008 Dec 23.
Artigo
em Inglês
| MEDLINE | ID: mdl-19049391
15.
ABC efflux pump-based resistance to chemotherapy drugs.
Chem Rev
; 109(7): 2989-3011, 2009 Jul.
Artigo
em Inglês
| MEDLINE | ID: mdl-19583429
16.
The reconstituted P-glycoprotein multidrug transporter is a flippase for glucosylceramide and other simple glycosphingolipids.
Biochem J
; 389(Pt 2): 517-26, 2005 Jul 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-15799713
17.
Functional reconstitution and channel activity measurements of purified wildtype and mutant CFTR protein.
J Vis Exp
; (97)2015 Mar 09.
Artigo
em Inglês
| MEDLINE | ID: mdl-25867140
18.
VX-809 and related corrector compounds exhibit secondary activity stabilizing active F508del-CFTR after its partial rescue to the cell surface.
Chem Biol
; 21(5): 666-78, 2014 May 22.
Artigo
em Inglês
| MEDLINE | ID: mdl-24726831
19.
Proton-dependent gating and proton uptake by Wzx support O-antigen-subunit antiport across the bacterial inner membrane.
mBio
; 4(5): e00678-13, 2013 Sep 10.
Artigo
em Inglês
| MEDLINE | ID: mdl-24023388
20.
Identification and validation of hits from high throughput screens for CFTR modulators.
Curr Pharm Des
; 18(5): 628-41, 2012.
Artigo
em Inglês
| MEDLINE | ID: mdl-22229556