Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip
Background Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene
encoding the cystic fibrosis transmembrane conductance regulator (CFTR), which results in …
encoding the cystic fibrosis transmembrane conductance regulator (CFTR), which results in …
From static to dynamic: a review on the role of mucus heterogeneity in particle and microbial transport
DD Pednekar, MA Liguori, CNH Marques… - ACS Biomaterials …, 2022 - ACS Publications
Mucus layers (McLs) are on the front line of the human defense system that protect us from
foreign abiotic/biotic particles (eg, airborne virus SARS-CoV-2) and lubricates our organs …
foreign abiotic/biotic particles (eg, airborne virus SARS-CoV-2) and lubricates our organs …
Animal models of cystic fibrosis in the era of highly effective modulator therapies
BR Grubb, A Livraghi-Butrico - Current opinion in pharmacology, 2022 - Elsevier
Few human genetic diseases can rely on the availability of as many and as diverse animal
models as cystic fibrosis (CF), a multiorgan syndrome caused by functional absence of cystic …
models as cystic fibrosis (CF), a multiorgan syndrome caused by functional absence of cystic …
Dynamic regulation of airway surface liquid pH by TMEM16A and SLC26A4 in cystic fibrosis nasal epithelia with rare mutations
L Delpiano, LW Rodenburg, M Burke… - Proceedings of the …, 2023 - National Acad Sciences
In cystic fibrosis (CF), defects in the CF transmembrane conductance regulator (CFTR)
channel lead to an acidic airway surface liquid (ASL), which compromises innate defence …
channel lead to an acidic airway surface liquid (ASL), which compromises innate defence …
TMEM16A chloride channel does not drive mucus production
Airway mucus obstruction is the main cause of morbidity in cystic fibrosis, a disease caused
by mutations in the CFTR Cl− channel. Activation of non-CFTR Cl− channels such as …
by mutations in the CFTR Cl− channel. Activation of non-CFTR Cl− channels such as …
Viral vectors, animal models, and cellular targets for gene therapy of cystic fibrosis lung disease
Y Tang, Z Yan, JF Engelhardt - Human gene therapy, 2020 - liebertpub.com
After more than two decades since clinical trials tested the first use of recombinant adeno-
associated virus (rAAV) to treat cystic fibrosis (CF) lung disease, gene therapy for this …
associated virus (rAAV) to treat cystic fibrosis (CF) lung disease, gene therapy for this …
Factoring in the Complexity of the Cystic Fibrosis Lung to Understand Aspergillus fumigatus and Pseudomonas aeruginosa Interactions
Pseudomonas aeruginosa has long been established as the most prevalent respiratory
pathogen in Cystic Fibrosis (CF) patients, with opportunistic infection causing profound …
pathogen in Cystic Fibrosis (CF) patients, with opportunistic infection causing profound …
An ex vivo rat trachea model reveals abnormal airway physiology and a gland secretion defect in cystic fibrosis
E Harris, M Easter, J Ren, S Krick, J Barnes, SM Rowe - Plos one, 2023 - journals.plos.org
Cystic fibrosis (CF) is a genetic disease hallmarked by aberrant ion transport that results in
delayed mucus clearance, chronic infection, and progressive lung function decline. Several …
delayed mucus clearance, chronic infection, and progressive lung function decline. Several …
Role of inflammation and oxidative stress in tissue damage associated with cystic fibrosis: CAPE as a future therapeutic strategy
VEM Soares, TIT do Carmo, F Dos Anjos… - Molecular and Cellular …, 2022 - Springer
Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic
fibrosis transmembrane conductance regulator (CFTR) gene, responsible for the synthesis …
fibrosis transmembrane conductance regulator (CFTR) gene, responsible for the synthesis …
Normal murine respiratory tract has its mucus concentrated in clouds based on the Muc5b mucin
D Fakih, AM Rodriguez-Piñeiro… - … of Physiology-Lung …, 2020 - journals.physiology.org
The organization of the normal airway mucus system differs in small experimental animals
from that in humans and large mammals. To address normal murine airway mucociliary …
from that in humans and large mammals. To address normal murine airway mucociliary …