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 …
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 …
continuous cycles of infection, particularly with Pseudomonas aeruginosa and …
Mucociliary clearance in cystic fibrosis knockout mice infected with Pseudomonas aeruginosa
In this study, we examined whether mucociliary clearance differed between cystic fibrosis
(CF) knockout mice and wildtype controls. Additionally, we investigated whether infection …
(CF) knockout mice and wildtype controls. Additionally, we investigated whether infection …
Mucus aberrant properties in CF: Insights from cells and animal models
Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic
fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus …
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 …
predisposes airways to bacterial infection. Advanced CF is characterized by a deficit in …
[HTML][HTML] Progress in understanding mucus abnormalities in cystic fibrosis airways
Normal airways below the carina maintain an essentially sterile environment via a multi-
pronged innate defence system that includes mucus clearance via mucociliary clearance …
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 …
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 …
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 …
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 …
defect in cystic fibrosis (CF) remain largely unclear. New animal models of CF, coupled with …