Detalhe da pesquisa
1.
Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles.
Am J Respir Crit Care Med
; 205(5): 540-549, 2022 03 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-34936849
2.
Lack of IL-1 Receptor Signaling Reduces Spontaneous Airway Eosinophilia in Juvenile Mice with Muco-Obstructive Lung Disease.
Am J Respir Cell Mol Biol
; 62(3): 300-309, 2020 03.
Artigo
em Inglês
| MEDLINE | ID: mdl-31499011
3.
Effects of Lumacaftor-Ivacaftor Therapy on Cystic Fibrosis Transmembrane Conductance Regulator Function in Phe508del Homozygous Patients with Cystic Fibrosis.
Am J Respir Crit Care Med
; 197(11): 1433-1442, 2018 06 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-29327948
4.
Impaired mucus clearance exacerbates allergen-induced type 2 airway inflammation in juvenile mice.
J Allergy Clin Immunol
; 140(1): 190-203.e5, 2017 Jul.
Artigo
em Inglês
| MEDLINE | ID: mdl-27865862
5.
Comparison of Organoid Swelling and In Vivo Biomarkers of CFTR Function to Determine Effects of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation.
Am J Respir Crit Care Med
; 202(11): 1589-1592, 2020 12 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-32687398
6.
Hypoxic epithelial necrosis triggers neutrophilic inflammation via IL-1 receptor signaling in cystic fibrosis lung disease.
Am J Respir Crit Care Med
; 191(8): 902-13, 2015 Apr 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-25607238
7.
Lack of neutrophil elastase reduces inflammation, mucus hypersecretion, and emphysema, but not mucus obstruction, in mice with cystic fibrosis-like lung disease.
Am J Respir Crit Care Med
; 189(9): 1082-92, 2014 May 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-24678594
8.
Airway mucus obstruction triggers macrophage activation and matrix metalloproteinase 12-dependent emphysema.
Am J Respir Cell Mol Biol
; 51(5): 709-20, 2014 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-24828142
9.
Hypertonic saline is effective in the prevention and treatment of mucus obstruction, but not airway inflammation, in mice with chronic obstructive lung disease.
Am J Respir Cell Mol Biol
; 49(3): 410-7, 2013 Sep.
Artigo
em Inglês
| MEDLINE | ID: mdl-23590312
10.
Effects of lumacaftor-ivacaftor therapy on cystic fibrosis transmembrane conductance regulator function in F508del homozygous patients with cystic fibrosis aged 2-11 years.
Front Pharmacol
; 14: 1188051, 2023.
Artigo
em Inglês
| MEDLINE | ID: mdl-37324488
11.
Intestinal Current Measurements Detect Activation of Mutant CFTR in Patients with Cystic Fibrosis with the G551D Mutation Treated with Ivacaftor.
Am J Respir Crit Care Med
; 192(10): 1252-5, 2015 Nov 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-26568242
12.
Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice.
Am J Respir Crit Care Med
; 178(12): 1245-56, 2008 Dec 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-18849497
13.
Assessment of CFTR function in rectal biopsies for the diagnosis of cystic fibrosis.
J Cyst Fibros
; 3 Suppl 2: 165-9, 2004 Aug.
Artigo
em Inglês
| MEDLINE | ID: mdl-15463952
14.
CFTR regulates early pathogenesis of chronic obstructive lung disease in ßENaC-overexpressing mice.
PLoS One
; 7(8): e44059, 2012.
Artigo
em Inglês
| MEDLINE | ID: mdl-22937152
15.
The K+ channel opener 1-EBIO potentiates residual function of mutant CFTR in rectal biopsies from cystic fibrosis patients.
PLoS One
; 6(8): e24445, 2011.
Artigo
em Inglês
| MEDLINE | ID: mdl-21909392
16.
Activation of ion secretion via proteinase-activated receptor-2 in human colon.
Am J Physiol Gastrointest Liver Physiol
; 282(2): G200-10, 2002 Feb.
Artigo
em Inglês
| MEDLINE | ID: mdl-11804840
17.
The DeltaF508 mutation results in loss of CFTR function and mature protein in native human colon.
Gastroenterology
; 126(1): 32-41, 2004 Jan.
Artigo
em Inglês
| MEDLINE | ID: mdl-14699484
18.
CFTR Cl- channel function in native human colon correlates with the genotype and phenotype in cystic fibrosis.
Gastroenterology
; 127(4): 1085-95, 2004 Oct.
Artigo
em Inglês
| MEDLINE | ID: mdl-15480987