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 …

Pathophysiology and management of pulmonary infections in cystic fibrosis

RL Gibson, JL Burns, BW Ramsey - American journal of respiratory …, 2003 - atsjournals.org
This comprehensive State of the Art review summarizes the current published knowledge
base regarding the pathophysiology and microbiology of pulmonary disease in cystic …

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 …

Exacerbations in cystic fibrosis· 1: epidemiology and pathogenesis

CH Goss, JL Burns - Thorax, 2007 - thorax.bmj.com
With the improving survival of patients with cystic fibrosis (CF), the clinical spectrum of this
complex multisystem disease continues to evolve. One of the most important clinical events …

From mucins to mucus: toward a more coherent understanding of this essential barrier

DJ Thornton, JK Sheehan - Proceedings of the American Thoracic …, 2004 - atsjournals.org
Mucus is essential for protection of the airways; however, in chronic airway disease mucus
hypersecretion is an important factor in morbidity and mortality. The properties of the mucus …

CFTR, mucins, and mucus obstruction in cystic fibrosis

SM Kreda, CW Davis, MC Rose - Cold Spring …, 2012 - perspectivesinmedicine.cshlp.org
Mucus pathology in cystic fibrosis (CF) has been known for as long as the disease has been
recognized and is sometimes called mucoviscidosis. The disease is marked by mucus …

Normal and cystic fibrosis airway surface liquid homeostasis: the effects of phasic shear stress and viral infections

R Tarran, B Button, M Picher, AM Paradiso… - Journal of Biological …, 2005 - ASBMB
Mammalian airways normally regulate the volume of a thin liquid layer, the periciliary liquid
(PCL), to facilitate the mucus clearance component of lung defense. Studies under standard …

The porcine lung as a potential model for cystic fibrosis

CS Rogers, WM Abraham… - … of Physiology-Lung …, 2008 - journals.physiology.org
Airway disease currently causes most of the morbidity and mortality in patients with cystic
fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing …

Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography

L Liu, KK Chu, GH Houser, BJ Diephuis, Y Li… - PloS one, 2013 - journals.plos.org
We demonstrate the use of a high resolution form of optical coherence tomography, termed
micro-OCT (μOCT), for investigating the functional microanatomy of airway epithelia. μOCT …