[HTML][HTML] Evidence of early increased sialylation of airway mucins and defective mucociliary clearance in CFTR-deficient piglets
I Caballero, B Ringot-Destrez, M Si-Tahar… - Journal of Cystic …, 2021 - Elsevier
Background Bacterial colonization in cystic fibrosis (CF) lungs has been directly associated
to the loss of CFTR function, and/or secondarily linked to repetitive cycles of chronic …
to the loss of CFTR function, and/or secondarily linked to repetitive cycles of chronic …
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 …
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 …
Mucociliary transport in healthy and cystic fibrosis pig airways
Y Xie, L Ostedgaard, MH Abou Alaiwa, L Lu… - Annals of the …, 2018 - atsjournals.org
Cystic fibrosis (CF) lung disease is the major cause of morbidity and mortality in people with
CF. Abnormal mucociliary transport has been the leading hypothesis for the underlying …
CF. Abnormal mucociliary transport has been the leading hypothesis for the underlying …
[HTML][HTML] Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs
NS Joo, HJ Cho, M Khansaheb… - The Journal of clinical …, 2010 - Am Soc Clin Investig
Cystic fibrosis (CF) results from mutations that disrupt CF transmembrane conductance
regulator (CFTR), an anion channel found mainly in apical membranes of epithelial cells. CF …
regulator (CFTR), an anion channel found mainly in apical membranes of epithelial cells. CF …
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 …
Primary inflammation in human cystic fibrosis small airways
R Tirouvanziam, I Khazaal… - American Journal of …, 2002 - journals.physiology.org
Most cystic fibrosis (CF) patients die of lung failure, due to the combined effects of bacterial
infection, neutrophil-mediated inflammation, and airway obstruction by hyperviscous mucus …
infection, neutrophil-mediated inflammation, and airway obstruction by hyperviscous mucus …
[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 …
Host mucin glycosylation plays a role in bacterial adhesion in lungs of individuals with cystic fibrosis
V Venkatakrishnan, NH Packer… - Expert Review of …, 2013 - Taylor & Francis
Malfunction of the cell surface glycoprotein, cystic fibrosis transmembrane conductance
regulator, is the molecular hallmark of cystic fibrosis (CF), causing salt imbalance across the …
regulator, is the molecular hallmark of cystic fibrosis (CF), causing salt imbalance across the …
Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator–knockout ferret lungs
Mucociliary clearance (MCC) and submucosal glands are major components of airway
innate immunity that have impaired function in cystic fibrosis (CF). Although both of these …
innate immunity that have impaired function in cystic fibrosis (CF). Although both of these …