Physiology and pathophysiology of human airway mucus

DB Hill, B Button, M Rubinstein… - Physiological …, 2022 - journals.physiology.org
The mucus clearance system is the dominant mechanical host defense system of the human
lung. Mucus is cleared from the lung by cilia and airflow, including both two-phase gas-liquid …

Role of CFTR in epithelial physiology

V Saint-Criq, MA Gray - Cellular and Molecular Life Sciences, 2017 - Springer
Salt and fluid absorption and secretion are two processes that are fundamental to epithelial
function and whole body fluid homeostasis, and as such are tightly regulated in epithelial …

CFTR protein: not just a chloride channel?

LS Hanssens, J Duchateau, GJ Casimir - Cells, 2021 - mdpi.com
Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in a gene encoding
a protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). The CFTR …

Tight junctions in pulmonary epithelia during lung inflammation

OH Wittekindt - Pflügers Archiv-European Journal of Physiology, 2017 - Springer
Inflammatory lung diseases like asthma bronchiale, chronic obstructive pulmonary disease
and allergic airway inflammation are widespread public diseases that constitute an …

CFTR: cystic fibrosis and beyond

MA Mall, D Hartl - 2014 - Eur Respiratory Soc
Cystic fibrosis (CF) remains the most common fatal hereditary lung disease. The discovery of
the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the …

Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …

Airway surface liquid pH regulation in airway epithelium current understandings and gaps in knowledge

M Zajac, E Dreano, A Edwards, G Planelles… - International journal of …, 2021 - mdpi.com
Knowledge on the mechanisms of acid and base secretion in airways has progressed
recently. The aim of this review is to summarize the known mechanisms of airway surface …

The Concise Guide to PHARMACOLOGY 2013/14: ion channels

SPH Alexander, HE Benson… - British journal of …, 2013 - Wiley Online Library
Abstract The Concise Guide to PHARMACOLOGY 2013/14 provides concise overviews of
the key properties of over 2000 human drug targets with their pharmacology, plus links to an …

Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targets

IJ Haq, MA Gray, JP Garnett, C Ward, M Brodlie - Thorax, 2016 - thorax.bmj.com
Cystic fibrosis (CF) is a life-limiting disease characterised by recurrent respiratory infections,
inflammation and lung damage. The volume and composition of the airway surface liquid …

Molecular basis for pH-dependent mucosal dehydration in cystic fibrosis airways

AL Garland, WG Walton, RD Coakley… - Proceedings of the …, 2013 - National Acad Sciences
The ability to maintain proper airway surface liquid (ASL) volume homeostasis is vital for
mucus hydration and clearance, which are essential aspects of the mammalian lung's innate …