[HTML][HTML] Invited review: human air-liquid-interface organotypic airway tissue models derived from primary tracheobronchial epithelial cells—overview and perspectives

X Cao, JP Coyle, R Xiong, Y Wang, RH Heflich… - In Vitro Cellular & …, 2021 - Springer
The lung is an organ that is directly exposed to the external environment. Given the large
surface area and extensive ventilation of the lung, it is prone to exposure to airborne …

Anatomy and bronchoscopy of the porcine lung. A model for translational respiratory medicine

EP Judge, JML Hughes, JJ Egan… - American journal of …, 2014 - atsjournals.org
The porcine model has contributed significantly to biomedical research over many decades.
The similar size and anatomy of pig and human organs make this model particularly …

[HTML][HTML] Animal and cell culture models for cystic fibrosis: which model is right for your application?

A McCarron, D Parsons, M Donnelley - The American journal of pathology, 2021 - Elsevier
Over the past 30 years, a range of cystic fibrosis (CF) animal models have been generated
for research purposes. Different species, including mice, rats, ferrets, rabbits, pigs, sheep …

[HTML][HTML] Gene therapy for cystic fibrosis: new tools for precision medicine

JA Lee, A Cho, EN Huang, Y Xu, H Quach, J Hu… - Journal of Translational …, 2021 - Springer
The discovery of the Cystic fibrosis (CF) gene in 1989 has paved the way for incredible
progress in treating the disease such that the mean survival age of individuals living with CF …

[HTML][HTML] A sheep model of cystic fibrosis generated by CRISPR/Cas9 disruption of the CFTR gene

Z Fan, IV Perisse, CU Cotton, M Regouski, Q Meng… - JCI insight, 2018 - ncbi.nlm.nih.gov
Cystic fibrosis (CF) is a genetic disease caused by mutations in the CF transmembrane
conductance regulator (CFTR) gene. The major cause of limited life span in CF patients is …

[HTML][HTML] Animal models in the pathophysiology of cystic fibrosis

A Semaniakou, RP Croll, V Chappe - Frontiers in pharmacology, 2019 - frontiersin.org
Our understanding of the multiorgan pathology of cystic fibrosis (CF) has improved
impressively during the last decades, but we still lack a full comprehension of the disease …

[HTML][HTML] Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia

JH Chen, DA Stoltz, PH Karp, SE Ernst, AA Pezzulo… - Cell, 2010 - cell.com
Defective transepithelial electrolyte transport is thought to initiate cystic fibrosis (CF) lung
disease. Yet, how loss of CFTR affects electrolyte transport remains uncertain. CFTR−/− pigs …

The porcine lung as a potential model for cystic fibrosis

CS Rogers, WM Abraham… - … of Physiology-Lung …, 2008 - journals.physiology.org
Airway disease currently causes most of the morbidity and mortality in patients with cystic
fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing …

Animal models of cystic fibrosis pathology: phenotypic parallels and divergences

GM Lavelle, MM White, N Browne… - BioMed research …, 2016 - Wiley Online Library
Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) gene. The resultant characteristic ion transport defect results in decreased …

[HTML][HTML] Airway disease phenotypes in animal models of cystic fibrosis

A McCarron, M Donnelley, D Parsons - Respiratory research, 2018 - Springer
In humans, cystic fibrosis (CF) lung disease is characterised by chronic infection,
inflammation, airway remodelling, and mucus obstruction. A lack of pulmonary …