Airway gland structure and function

JH Widdicombe, JJ Wine - Physiological reviews, 2015 - journals.physiology.org
Submucosal glands contribute to airway surface liquid (ASL), a film that protects all airway
surfaces. Glandular mucus comprises electrolytes, water, the gel-forming mucin MUC5B …

Porcine models of digestive disease: the future of large animal translational research

LM Gonzalez, AJ Moeser, AT Blikslager - Translational research, 2015 - Elsevier
There is increasing interest in nonrodent translational models for the study of human
disease. The pig, in particular, serves as a useful animal model for the study of …

Coordinated international action to accelerate genome-to-phenome with FAANG, the Functional Annotation of Animal Genomes project

FAANG Consortium, L Andersson, AL Archibald… - Genome biology, 2015 - Springer
Coordinated international action to accelerate genome-to-phenome with FAANG, the
Functional Annotation of Animal Genomes project | Genome Biology Skip to main content …

Disruption of the CFTR Gene Produces a Model of Cystic Fibrosis in Newborn Pigs

CS Rogers, DA Stoltz, DK Meyerholz, LS Ostedgaard… - Science, 2008 - science.org
Almost two decades after CFTR was identified as the gene responsible for cystic fibrosis
(CF), we still lack answers to many questions about the pathogenesis of the disease, and it …

Arteriovenous metabolomics in pigs reveals CFTR regulation of metabolism in multiple organs

H Bae, BR Kim, S Jung, J Le, D van der Heide… - The Journal of clinical …, 2024 - jci.org
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause
cystic fibrosis (CF), a multiorgan disease that is characterized by diverse metabolic defects …

Airway acidification initiates host defense abnormalities in cystic fibrosis mice

VS Shah, DK Meyerholz, XX Tang, L Reznikov… - Science, 2016 - science.org
Cystic fibrosis (CF) is caused by mutations in the gene that encodes the cystic fibrosis
transmembrane conductance regulator (CFTR) anion channel. In humans and pigs, the loss …

Generation of multipotent lung and airway progenitors from mouse ESCs and patient-specific cystic fibrosis iPSCs

H Mou, R Zhao, R Sherwood, T Ahfeldt, A Lapey… - Cell stem cell, 2012 - cell.com
Deriving lung progenitors from patient-specific pluripotent cells is a key step in producing
differentiated lung epithelium for disease modeling and transplantation. By mimicking the …

Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis

X Sun, H Sui, JT Fisher, Z Yan, X Liu… - The Journal of …, 2010 - Am Soc Clin Investig
Cystic fibrosis (CF) is a recessive disease that affects multiple organs. It is caused by
mutations in CFTR. Animal modeling of this disease has been challenging, with species-and …

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 …

The cystic fibrosis intestine

RC De Lisle, D Borowitz - Cold Spring …, 2013 - perspectivesinmedicine.cshlp.org
The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis
transmembrane regulator protein (CFTR). The majority of people with CF have a limited life …