Airway submucosal glands from cystic fibrosis swine suffer from abnormal ion transport across the serous acini, collecting duct and ciliated duct 2

PG Hassan, AM Shipley, TE Machen, JP Ianowski - journals.physiology.org
The human airway is protected by an efficient innate defence mechanism that requires 28
healthy secretion of airway surface liquid (ASL) to clear pathogens from the lungs. Most 29 …

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 …

[HTML][HTML] 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 …

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 …

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 …

[HTML][HTML] The Big Impact of Small Airway pH

K Okuda, SH Randell, SE Birket - American Journal of Respiratory …, 2021 - atsjournals.org
Cystic fibrosis (CF) lung disease, caused by abnormal ion transport because of deficient
CFTR (cystic fibrosis transmembrane conductance regulator) function, is characterized by a …

Interleukin‐8 stimulates CFTR‐mediated mucus secretion by submucosal glands in swine (869.10)

H Hansvall, J Ianowski - The FASEB Journal, 2014 - Wiley Online Library
Cystic Fibrosis (CF) is an autosomal recessive condition caused by mutations in the gene
encoding the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) anion …

Abnormal respiratory epithelial ion transport in cystic fibrosis

MR Knowles, MJ Stutts, JR Yankaskas, JT Gatzy… - Clinics in chest …, 1986 - Elsevier
Abnormal airway secretions characterize asthma" and cystic fibrosis (CF), 13.73 obstructive
airway diseases with different clinical courses. Whereas airway secretions that are retained …

[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 …