Mucin production and hydration responses to mucopurulent materials in normal versus cystic fibrosis airway epithelia
LH Abdullah, R Coakley, MJ Webster… - American journal of …, 2018 - atsjournals.org
Rationale: Cystic fibrosis (CF) airways disease produces a mucoobstructive lung phenotype
characterized by airways mucus plugging, epithelial mucous cell metaplasia/hyperplasia …
characterized by airways mucus plugging, epithelial mucous cell metaplasia/hyperplasia …
Cystic fibrosis and the relationship between mucin and chloride secretion by cultures of human airway gland mucous cells
WE Finkbeiner, LT Zlock, M Morikawa… - … of Physiology-Lung …, 2011 - journals.physiology.org
—We investigated how cystic fibrosis (CF) alters the relationship between Cl− and mucin
secretion in cultures of non-CF and CF human tracheobronchial gland mucous (HTGM and …
secretion in cultures of non-CF and CF human tracheobronchial gland mucous (HTGM and …
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 …
Cystic fibrosis airway secretions exhibit mucin hyperconcentration and increased osmotic pressure
The pathogenesis of mucoinfective lung disease in cystic fibrosis (CF) patients likely
involves poor mucus clearance. A recent model of mucus clearance predicts that mucus flow …
involves poor mucus clearance. A recent model of mucus clearance predicts that mucus flow …
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 …
MUC5AC and MUC5B mucins increase in cystic fibrosis airway secretions during pulmonary exacerbation
MO Henke, G John, M Germann… - American journal of …, 2007 - atsjournals.org
Rationale: Cystic fibrosis (CF) is believed to be associated with mucus hypersecretion; thus,
the principal airway gel-forming mucins, MUC5AC and MUC5B, are also expected to be …
the principal airway gel-forming mucins, MUC5AC and MUC5B, are also expected to be …
Cystic fibrosis airway mucus hyperconcentration produces a vicious cycle of mucin, pathogen, and inflammatory interactions that promotes disease persistence
BD Batson, BT Zorn, G Radicioni… - American Journal of …, 2022 - atsjournals.org
The dynamics describing the vicious cycle characteristic of cystic fibrosis (CF) lung disease,
initiated by stagnant mucus and perpetuated by infection and inflammation, remain unclear …
initiated by stagnant mucus and perpetuated by infection and inflammation, remain unclear …
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 …
CFTR, mucins, and mucus obstruction in cystic fibrosis
SM Kreda, CW Davis, MC Rose - Cold Spring …, 2012 - perspectivesinmedicine.cshlp.org
Mucus pathology in cystic fibrosis (CF) has been known for as long as the disease has been
recognized and is sometimes called mucoviscidosis. The disease is marked by mucus …
recognized and is sometimes called mucoviscidosis. The disease is marked by mucus …
Mucus concentration–dependent biophysical abnormalities unify submucosal gland and superficial airway dysfunction in cystic fibrosis
Cystic fibrosis (CF) is characterized by abnormal transepithelial ion transport. However, a
description of CF lung disease pathophysiology unifying superficial epithelial and …
description of CF lung disease pathophysiology unifying superficial epithelial and …