[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 …

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 …

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 …

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 …

[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 …

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 …

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 …

[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 …

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 …

Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator–knockout ferret lungs

NW Keiser, SE Birket, IA Evans, SR Tyler… - American journal of …, 2015 - atsjournals.org
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 …