Early alterations in airway mucociliary clearance and inflammation of the lamina propria in CF mice

JM Zahm, D Gaillard, F Dupuit… - … of Physiology-Cell …, 1997 - journals.physiology.org
In cystic fibrosis (CF), whether cystic fibrosis transmembrane conductance regulator (CFTR)
dysfunction leads to decreased mucociliary clearance and mucus hypersecretion, before …

The relationship of chronic mucin secretion to airway disease in normal and CFTR-deficient mice

VL Cressman, EM Hicks, WK Funkhouser… - American journal of …, 1998 - atsjournals.org
In the cystic fibrosis (CF) patient, lung function decreases throughout life as a result of
continuous cycles of infection, particularly with Pseudomonas aeruginosa and …

Mucociliary clearance in cystic fibrosis knockout mice infected with Pseudomonas aeruginosa

EA Cowley, CG Wang, D Gosselin… - European …, 1997 - Eur Respiratory Soc
In this study, we examined whether mucociliary clearance differed between cystic fibrosis
(CF) knockout mice and wildtype controls. Additionally, we investigated whether infection …

Mucus aberrant properties in CF: Insights from cells and animal models

C Ehre, GC Hansson, DJ Thornton… - Journal of Cystic …, 2023 - Elsevier
Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic
fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus …

Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis

MJ Hoegger, AJ Fischer, JD McMenimen… - Science, 2014 - science.org
Lung disease in people with cystic fibrosis (CF) is initiated by defective host defense that
predisposes airways to bacterial infection. Advanced CF is characterized by a deficit in …

[HTML][HTML] Progress in understanding mucus abnormalities in cystic fibrosis airways

JJ Wine, GC Hansson, P König, NS Joo… - Journal of Cystic …, 2018 - Elsevier
Normal airways below the carina maintain an essentially sterile environment via a multi-
pronged innate defence system that includes mucus clearance via mucociliary clearance …

Static mucus impairs bacterial clearance and allows chronic infection with Pseudomonas aeruginosa in the cystic fibrosis rat

AG Henderson, JM Davis, JD Keith… - European …, 2022 - Eur Respiratory Soc
Cystic fibrosis airway disease is characterised by chronic Pseudomonas aeruginosa
infection. Successful eradication strategies have been hampered by a poor understanding of …

Airway mucus in cystic fibrosis

E Puchelle, O Bajolet, M Abély - Paediatric respiratory reviews, 2002 - Elsevier
Defective expression and function of the cystic fibrosis transmembrane conductance
regulator (CFTR) in cystic fibrosis (CF) airway epithelial cells are associated with airway …

Mucin glycosylation and sulphation in airway epithelial cells is not influenced by cystic fibrosis transmembrane conductance regulator expression

SH Leir, S Parry, T Palmai-Pallag, J Evans… - American journal of …, 2005 - atsjournals.org
Abnormalities in mucus properties and clearance make a major contribution to the pathology
of cystic fibrosis (CF). Our aim was to test the hypothesis that the defects in CF mucus are a …

[HTML][HTML] Development of an airway mucus defect in the cystic fibrosis rat

SE Birket, JM Davis, CM Fernandez, KL Tuggle… - JCI insight, 2018 - ncbi.nlm.nih.gov
The mechanisms underlying the development and natural progression of the airway mucus
defect in cystic fibrosis (CF) remain largely unclear. New animal models of CF, coupled with …