Detalhe da pesquisa
1.
Engineered tRNAs suppress nonsense mutations in cells and in vivo.
Nature
; 618(7966): 842-848, 2023 Jun.
Artigo
Inglês
| MEDLINE | ID: mdl-37258671
2.
Transgenic ferret models define pulmonary ionocyte diversity and function.
Nature
; 621(7980): 857-867, 2023 Sep.
Artigo
Inglês
| MEDLINE | ID: mdl-37730992
3.
Human distal airways contain a multipotent secretory cell that can regenerate alveoli.
Nature
; 604(7904): 120-126, 2022 04.
Artigo
Inglês
| MEDLINE | ID: mdl-35355013
4.
Pulmonary Fibrosis Stakeholder Summit: A Joint NHLBI, Three Lakes Foundation, and Pulmonary Fibrosis Foundation Workshop Report.
Am J Respir Crit Care Med
; 209(4): 362-373, 2024 Feb 15.
Artigo
Inglês
| MEDLINE | ID: mdl-38113442
5.
Triple Therapy for Cystic Fibrosis Phe508del-Gating and -Residual Function Genotypes.
N Engl J Med
; 385(9): 815-825, 2021 08 26.
Artigo
Inglês
| MEDLINE | ID: mdl-34437784
6.
A revised airway epithelial hierarchy includes CFTR-expressing ionocytes.
Nature
; 560(7718): 319-324, 2018 08.
Artigo
Inglês
| MEDLINE | ID: mdl-30069044
7.
The synthetic aminoglycoside ELX-02 induces readthrough of G550X-CFTR producing superfunctional protein that can be further enhanced by CFTR modulators.
Am J Physiol Lung Cell Mol Physiol
; 324(6): L756-L770, 2023 06 01.
Artigo
Inglês
| MEDLINE | ID: mdl-37014818
8.
Mucociliary clearance augmenting drugs block SARS-CoV-2 replication in human airway epithelial cells.
Am J Physiol Lung Cell Mol Physiol
; 324(4): L493-L506, 2023 04 01.
Artigo
Inglês
| MEDLINE | ID: mdl-36809189
9.
Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond.
Eur Respir J
; 61(4)2023 04.
Artigo
Inglês
| MEDLINE | ID: mdl-37003609
10.
Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from a 192-week open-label extension study.
Eur Respir J
; 62(6)2023 12.
Artigo
Inglês
| MEDLINE | ID: mdl-37945033
11.
Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis: A Clinical Trial.
Am J Respir Crit Care Med
; 205(5): 529-539, 2022 03 01.
Artigo
Inglês
| MEDLINE | ID: mdl-34784492
12.
Triamterene Functions as an Effective Nonsense Suppression Agent for MPS I-H (Hurler Syndrome).
Int J Mol Sci
; 24(5)2023 Feb 24.
Artigo
Inglês
| MEDLINE | ID: mdl-36901952
13.
Preclinical evaluation of the epithelial sodium channel inhibitor AZD5634 and implications on human translation.
Am J Physiol Lung Cell Mol Physiol
; 323(5): L536-L547, 2022 11 01.
Artigo
Inglês
| MEDLINE | ID: mdl-36098422
14.
Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.
N Engl J Med
; 381(19): 1809-1819, 2019 11 07.
Artigo
Inglês
| MEDLINE | ID: mdl-31697873
15.
Inhaled gene therapy of preclinical muco-obstructive lung diseases by nanoparticles capable of breaching the airway mucus barrier.
Thorax
; 77(8): 812-820, 2022 08.
Artigo
Inglês
| MEDLINE | ID: mdl-34697091
16.
Poly (acetyl, arginyl) glucosamine disrupts Pseudomonas aeruginosa biofilms and enhances bacterial clearance in a rat lung infection model.
Microbiology (Reading)
; 168(1)2022 01.
Artigo
Inglês
| MEDLINE | ID: mdl-35077346
17.
Static mucus impairs bacterial clearance and allows chronic infection with Pseudomonas aeruginosa in the cystic fibrosis rat.
Eur Respir J
; 2022 Feb 03.
Artigo
Inglês
| MEDLINE | ID: mdl-35115338
18.
Evaluation of a novel CFTR potentiator in COPD ferrets with acquired CFTR dysfunction.
Eur Respir J
; 60(1)2022 07.
Artigo
Inglês
| MEDLINE | ID: mdl-34916262
19.
Comparative transcriptomics in human COPD reveals dysregulated genes uniquely expressed in ferrets.
Respir Res
; 23(1): 277, 2022 Oct 10.
Artigo
Inglês
| MEDLINE | ID: mdl-36217144
20.
Changes in Glucose Breath Test in Cystic Fibrosis Patients Treated With 1 Month of Lumacaftor/Ivacaftor.
J Pediatr Gastroenterol Nutr
; 75(1): 42-47, 2022 07 01.
Artigo
Inglês
| MEDLINE | ID: mdl-35442228