Modeling pulmonary cystic fibrosis in a human lung airway-on-a-chip

R Plebani, R Potla, M Soong, H Bai, Z Izadifar… - Journal of Cystic …, 2022 - Elsevier
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 …

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 …

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 …

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 …

TMEM16A chloride channel does not drive mucus production

FB Simões, MC Quaresma, LA Clarke… - Life science …, 2019 - life-science-alliance.org
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 …

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 …

Factoring in the Complexity of the Cystic Fibrosis Lung to Understand Aspergillus fumigatus and Pseudomonas aeruginosa Interactions

E Beswick, J Amich, S Gago - Pathogens, 2020 - mdpi.com
Pseudomonas aeruginosa has long been established as the most prevalent respiratory
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 …

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 …

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 …