Detalhe da pesquisa
1.
Theratyping cystic fibrosis patients to guide elexacaftor/tezacaftor/ivacaftor out-of-label prescription.
Eur Respir J
; 62(4)2023 10.
Artigo
em Inglês
| MEDLINE | ID: mdl-37696564
2.
Correlating genotype with phenotype using CFTR-mediated whole-cell Cl- currents in human nasal epithelial cells.
J Physiol
; 600(6): 1515-1531, 2022 03.
Artigo
em Inglês
| MEDLINE | ID: mdl-34761808
3.
Genetic deletion of keratin 8 corrects the altered bone formation and osteopenia in a mouse model of cystic fibrosis.
Hum Mol Genet
; 25(7): 1281-93, 2016 Apr 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-26769674
4.
Pseudomonas aeruginosa LasB protease impairs innate immunity in mice and humans by targeting a lung epithelial cystic fibrosis transmembrane regulator-IL-6-antimicrobial-repair pathway.
Thorax
; 73(1): 49-61, 2018 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-28790180
5.
Reclassifying inconclusive diagnosis for cystic fibrosis with new generation sweat test.
Eur Respir J
; 60(2)2022 08.
Artigo
em Inglês
| MEDLINE | ID: mdl-35777769
6.
Might Brushed Nasal Cells Be a Surrogate for CFTR Modulator Clinical Response?
Am J Respir Crit Care Med
; 199(1): 123-126, 2019 01 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-30326728
7.
Prime editing functionally corrects cystic fibrosis-causing CFTR mutations in human organoids and airway epithelial cells.
Cell Rep Med
; 5(5): 101544, 2024 May 21.
Artigo
em Inglês
| MEDLINE | ID: mdl-38697102
8.
Putting bicarbonate on the spot: pharmacological insights for CFTR correction in the airway epithelium.
Front Pharmacol
; 14: 1293578, 2023.
Artigo
em Inglês
| MEDLINE | ID: mdl-38149052
9.
Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children.
J Cyst Fibros
; 22(6): 1070-1079, 2023 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-37422433
10.
Primary Human Nasal Epithelial Cells: Biobanking in the Context of Precision Medicine.
J Vis Exp
; (182)2022 04 22.
Artigo
em Inglês
| MEDLINE | ID: mdl-35532277
11.
Isolation, cultivation, and application of primary respiratory epithelial cells obtained by nasal brushing, polyp samples, or lung explants.
STAR Protoc
; 3(2): 101419, 2022 06 17.
Artigo
em Inglês
| MEDLINE | ID: mdl-35664255
12.
Reclassifying inconclusive diagnosis after newborn screening for cystic fibrosis. Moving forward.
J Cyst Fibros
; 21(3): 448-455, 2022 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-34949556
13.
Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice.
Sci Rep
; 12(1): 6132, 2022 04 12.
Artigo
em Inglês
| MEDLINE | ID: mdl-35413967
14.
Sweat Chloride Testing and Nasal Potential Difference (NPD) Are Primary Outcome Parameters in Treatment with Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Modulators.
J Pers Med
; 11(8)2021 Jul 27.
Artigo
em Inglês
| MEDLINE | ID: mdl-34442373
15.
Antisense oligonucleotide-based drug development for Cystic Fibrosis patients carrying the 3849+10 kb C-to-T splicing mutation.
J Cyst Fibros
; 20(5): 865-875, 2021 09.
Artigo
em Inglês
| MEDLINE | ID: mdl-34226157
16.
New insights into structure and function of bis-phosphinic acid derivatives and implications for CFTR modulation.
Sci Rep
; 11(1): 6842, 2021 03 25.
Artigo
em Inglês
| MEDLINE | ID: mdl-33767236
17.
Unsolved severe chronic rhinosinusitis elucidated by extensive CFTR genotyping.
Clin Case Rep
; 7(11): 2128-2134, 2019 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-31788264
18.
Characterization of two rat models of cystic fibrosis-KO and F508del CFTR-Generated by Crispr-Cas9.
Animal Model Exp Med
; 2(4): 297-311, 2019 Dec.
Artigo
em Inglês
| MEDLINE | ID: mdl-31942562
19.
Predictive factors for lumacaftor/ivacaftor clinical response.
J Cyst Fibros
; 18(3): 368-374, 2019 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-30595473
20.
Author Correction: Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis.
Sci Rep
; 9(1): 17535, 2019 Nov 21.
Artigo
em Inglês
| MEDLINE | ID: mdl-31754179