Assessing mucociliary transport of single particles in vivo shows variable speed and preference for the ventral trachea in newborn pigs

MJ Hoegger, M Awadalla, E Namati… - Proceedings of the …, 2014 - National Acad Sciences
Mucociliary transport (MCT) is an innate defense mechanism that removes particulates,
noxious material, and microorganisms from the lung. Several airway diseases exhibit …

Reduced pH and chloride levels in exhaled breath condensate of patients with chronic cough

A Niimi, LT Nguyen, O Usmani, B Mann, KF Chung - Thorax, 2004 - thorax.bmj.com
Background: Increased hydrogen and reduced chloride ionic environments of the airways
are conducive to the stimulation of cough. However, the constituents of the local milieu of the …

Absent secretion to vasoactive intestinal peptide in cystic fibrosis airway glands

NS Joo, T Irokawa, JV Wu, RC Robbins… - Journal of Biological …, 2002 - ASBMB
We are testing the hypothesis that the malfunctioning of airway gland serous cells is a
component of cystic fibrosis (CF) airway disease. CF is caused by mutations that disrupt CF …

Glycosylation of sputum mucins is altered in cystic fibrosis patients

BL Schulz, AJ Sloane, LJ Robinson, SS Prasad… - …, 2007 - academic.oup.com
Cystic fibrosis (CF) is characterized by chronic lung infection and inflammation, with periods
of acute exacerbation causing severe and irreversible lung tissue damage. We used protein …

Contribution of α7 nicotinic receptor to airway epithelium dysfunction under nicotine exposure

K Maouche, K Medjber, JM Zahm… - Proceedings of the …, 2013 - National Acad Sciences
Loss or dysfunction of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR)
leads to impairment of airway mucus transport and to chronic lung diseases resulting in …

A small molecule CFTR inhibitor produces cystic fibrosis‐like submucosal gland fluid secretions in normal airways

JR Thiagarajah, Y Song, PM Haggie… - The FASEB …, 2004 - Wiley Online Library
Airway submucosal glands have been proposed as a primary site for initiating and
sustaining airway disease in cystic fibrosis (CF). However, it has been difficult to define the …

New horizons in the treatment of cystic fibrosis

AW Cuthbert - British journal of pharmacology, 2011 - Wiley Online Library
Cystic fibrosis (CF) is a lethal, recessive, genetic disease affecting approximately 1 in 2500
live births among Caucasians. The CF gene codes for a cAMP/PKA‐dependent, ATP …

Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: a mechanism for sodium hyperabsorption in cystic fibrosis

MM Myerburg, MB Butterworth, EE McKenna… - Journal of Biological …, 2006 - ASBMB
Efficient clearance of mucus and inhaled pathogens from the lung is dependent on an
optimal airway surface liquid (ASL) volume, which is maintained by the regulated transport …

Mucus concentration–dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis

T Kato, G Radicioni, MJ Papanikolas, GV Stoychev… - Science …, 2022 - science.org
Cystic fibrosis (CF) is characterized by abnormal transepithelial ion transport. However, a
description of CF lung disease pathophysiology unifying superficial epithelial and …

Acidic submucosal gland pH and elevated protein concentration produce abnormal cystic fibrosis mucus

Y Xie, L Lu, XX Tang, TO Moninger, TJ Huang… - Developmental cell, 2020 - cell.com
In response to respiratory insults, airway submucosal glands secrete copious mucus strands
to increase mucociliary clearance and protect the lung. However, in cystic fibrosis …