Detalhe da pesquisa
1.
Engineered tRNAs suppress nonsense mutations in cells and in vivo.
Nature
; 618(7966): 842-848, 2023 Jun.
Artigo
em Inglês
| MEDLINE | ID: mdl-37258671
2.
Elexacaftor/VX-445-mediated CFTR interactome remodeling reveals differential correction driven by mutation-specific translational dynamics.
J Biol Chem
; 299(10): 105242, 2023 Oct.
Artigo
em Inglês
| MEDLINE | ID: mdl-37690692
3.
Mechanisms by which the cystic fibrosis transmembrane conductance regulator may influence SARS-CoV-2 infection and COVID-19 disease severity.
FASEB J
; 37(11): e23220, 2023 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-37801035
4.
Positive epistasis between disease-causing missense mutations and silent polymorphism with effect on mRNA translation velocity.
Proc Natl Acad Sci U S A
; 118(4)2021 01 26.
Artigo
em Inglês
| MEDLINE | ID: mdl-33468668
5.
The CFTR P67L variant reveals a key role for N-terminal lasso helices in channel folding, maturation, and pharmacologic rescue.
J Biol Chem
; 296: 100598, 2021.
Artigo
em Inglês
| MEDLINE | ID: mdl-33781744
6.
Association of cystic fibrosis transmembrane conductance regulator with epithelial sodium channel subunits carrying Liddle's syndrome mutations.
Am J Physiol Lung Cell Mol Physiol
; 321(2): L308-L320, 2021 08 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-34037494
7.
Improved correction of F508del-CFTR biogenesis with a folding facilitator and an inhibitor of protein ubiquitination.
Bioorg Med Chem Lett
; 48: 128243, 2021 09 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-34246753
8.
Stability Prediction for Mutations in the Cytosolic Domains of Cystic Fibrosis Transmembrane Conductance Regulator.
J Chem Inf Model
; 61(4): 1762-1777, 2021 04 26.
Artigo
em Inglês
| MEDLINE | ID: mdl-33720715
9.
Highly Efficient Gene Editing of Cystic Fibrosis Patient-Derived Airway Basal Cells Results in Functional CFTR Correction.
Mol Ther
; 28(7): 1684-1695, 2020 07 08.
Artigo
em Inglês
| MEDLINE | ID: mdl-32402246
10.
Ivacaftor Reverses Airway Mucus Abnormalities in a Rat Model Harboring a Humanized G551D-CFTR.
Am J Respir Crit Care Med
; 202(9): 1271-1282, 2020 11 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-32584141
11.
Assessing cell-specific effects of genetic variations using tRNA microarrays.
BMC Genomics
; 20(Suppl 8): 549, 2019 Jul 16.
Artigo
em Inglês
| MEDLINE | ID: mdl-31307398
12.
Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect.
PLoS Biol
; 14(5): e1002462, 2016 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-27168400
13.
Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.
J Biol Chem
; 291(4): 1854-1865, 2016 Jan 22.
Artigo
em Inglês
| MEDLINE | ID: mdl-26627831
14.
Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications.
Am J Physiol Lung Cell Mol Physiol
; 311(4): L719-L733, 2016 Oct 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-27474090
15.
Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy.
Am J Physiol Lung Cell Mol Physiol
; 310(10): L928-39, 2016 05 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-26968770
16.
PRE-CLINICAL AND CLINICAL VALIDATION OF AN ANTI-CANCER MODALITY THAT ABLATES REFRACTORY, LOW GROWTH FRACTION TUMORS.
Trans Am Clin Climatol Assoc
; 127: 59-70, 2016.
Artigo
em Inglês
| MEDLINE | ID: mdl-28066038
17.
A functional anatomic defect of the cystic fibrosis airway.
Am J Respir Crit Care Med
; 190(4): 421-32, 2014 Aug 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-25029666
18.
S-palmitoylation regulates biogenesis of core glycosylated wild-type and F508del CFTR in a post-ER compartment.
Biochem J
; 459(2): 417-25, 2014 Apr 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-24475974
19.
An autoregulatory mechanism governing mucociliary transport is sensitive to mucus load.
Am J Respir Cell Mol Biol
; 51(4): 485-93, 2014 Oct.
Artigo
em Inglês
| MEDLINE | ID: mdl-24937762
20.
The silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunction.
FASEB J
; 27(11): 4630-45, 2013 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-23907436