CFTR, mucins, and mucus obstruction in cystic fibrosis

SM Kreda, CW Davis, MC Rose - Cold Spring …, 2012 - perspectivesinmedicine.cshlp.org
… Studies of the genetic disease cystic fibrosis (CF) have been central to much of our current
understanding of the function of the mucus barrier in health and its dysfunction in disease, …

Unplugging mucus in cystic fibrosis and chronic obstructive pulmonary disease

MA Mall - Annals of the American Thoracic Society, 2016 - atsjournals.org
… Airway mucus obstruction is a key feature of cystic fibrosis (CF) and chronic obstructive
pulmonary disease (COPD… Airway mucus obstruction is a key feature of cystic fibrosis and chronic …

Mucus structure and properties in cystic fibrosis

BK Rubin - Paediatric respiratory reviews, 2007 - Elsevier
… Since the first description of cystic fibrosis (CF) more than 50 years ago, it has been known
… airway mucus was based, in part, on recognition of the severe bowel obstruction due to …

Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
… In muco‐obstructive diseases like CF, restoring proper mucus viscoelasticity and clearance
in the lungs remain major goals. The advancement of CFTR correctors and modulators …

Mucus obstruction and inflammation in early cystic fibrosis lung disease: Emerging role of the IL‐1 signaling pathway

A Balázs, MA Mall - Pediatric pulmonology, 2019 - Wiley Online Library
… lung damage in people with cystic fibrosis (CF). However, mucus plugging and neutrophilic
… ‐like lung disease support the concept that mucus plugging per se can trigger inflammation …

Airway mucus in cystic fibrosis

E Puchelle, O Bajolet, M Abély - Paediatric respiratory reviews, 2002 - Elsevier
… ) in cystic fibrosis (CF) airway epithelial cells are associated with airway mucus hypersecretion…
, to severe airway obstruction with hyperviscous and adhesive airway mucus. Whether the …

Lack of neutrophil elastase reduces inflammation, mucus hypersecretion, and emphysema, but not mucus obstruction, in mice with cystic fibrosis–like lung disease

S Gehrig, J Duerr, M Weitnauer, CJ Wagner… - American journal of …, 2014 - atsjournals.org
… not mucus obstruction and … mucus obstruction in the absence of mucus hypersecretion
indicating that additional rehydration therapies may be necessary for effective treatment of mucus

Cystic fibrosis since 1938

PB Davis - American journal of respiratory and critical care …, 2006 - atsjournals.org
Cystic fibrosis (CF) was first recognized as a separate … disease of mucus plugging of the
glandular ducts, termed “cystic … CF usually consists of obstructive biliary cirrhosis, characterized …

Cystic fibrosis and other respiratory diseases of impaired mucus clearance

A Livraghi, SH Randell - Toxicologic pathology, 2007 - journals.sagepub.com
… Beyond obstruction of airflow, excessive mucus that fails to clear may form plaques and
plugs that can serve as a nidus for infection by opportunistic pathogens, especially facultative …

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 … dysfunction causes chronic mucus obstruction, neutrophilic inflammation …