Airway submucosal glands from cystic fibrosis swine suffer from abnormal ion transport across the serous acini, collecting duct, and ciliated duct

X Luan, JS Tam, S Jagadeeshan… - … of Physiology-Lung …, 2020 - journals.physiology.org
The human airway is protected by an efficient innate defense mechanism that requires
healthy secretion of airway surface liquid (ASL) to clear pathogens from the lungs. Most of …

Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease

AS Verkman, Y Song… - American Journal of …, 2003 - journals.physiology.org
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) protein, an epithelial chloride channel expressed in the airways, pancreas, testis …

Chloride secretion by cultures of pig tracheal gland cells

JH Widdicombe, RM Borthwell… - … of Physiology-Lung …, 2012 - journals.physiology.org
Malfunction of airway submucosal glands contributes to the pathology of cystic fibrosis (CF),
and cell cultures of CF human airway glands show defects in Cl− and water transport …

Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense

X Li, XX Tang, LG Vargas Buonfiglio… - … of Physiology-Lung …, 2016 - journals.physiology.org
While pathological and clinical data suggest that small airways are involved in early cystic
fibrosis (CF) lung disease development, little is known about how the lack of cystic fibrosis …

Why mouse airway submucosal gland serous cells do not secrete fluid in response to cAMP stimulation

RJ Lee, JK Foskett - Journal of Biological Chemistry, 2012 - ASBMB
Airway submucosal glands are important sites of cystic fibrosis transmembrane conductance
regulator (CFTR) chloride (Cl−) channel expression and fluid secretion in the airway …

Regulation of the depth and composition of airway surface liquid

JH Widdicombe - Journal of anatomy, 2002 - Wiley Online Library
The airways are lined with a film of liquid about 10 µm deep that is in two layers. Around the
cilia is the watery periciliary sol. Over this is a mucous blanket that traps inhaled particles …

[PDF][PDF] cAMP triggers Na+ absorption by distal airway surface epithelium in cystic fibrosis swine

X Luan, Y Le, S Jagadeeshan, B Murray, JL Carmalt… - Cell Reports, 2021 - cell.com
A controversial hypothesis pertaining to cystic fibrosis (CF) lung disease is that the CF
transmembrane conductance regulator (CFTR) channel fails to inhibit the epithelial Na+ …

Optical imaging of Ca2+‐evoked fluid secretion by murine nasal submucosal gland serous acinar cells

RJ Lee, MP Limberis, MF Hennessy… - The Journal of …, 2007 - Wiley Online Library
Airway submucosal glands are sites of high expression of the cystic fibrosis transmembrane
conductance regulator (CFTR) Cl− channel and contribute to fluid homeostasis in the lung …

Cellular and molecular architecture of submucosal glands in wild-type and cystic fibrosis pigs

W Yu, TO Moninger, AL Thurman… - Proceedings of the …, 2022 - National Acad Sciences
Submucosal glands (SMGs) protect lungs but can also contribute to disease. For example, in
cystic fibrosis (CF), SMGs produce abnormal mucus that disrupts mucociliary transport. CF is …

Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs

NS Joo, HJ Cho, M Khansaheb… - The Journal of clinical …, 2010 - Am Soc Clin Investig
Cystic fibrosis (CF) results from mutations that disrupt CF transmembrane conductance
regulator (CFTR), an anion channel found mainly in apical membranes of epithelial cells. CF …