Airway gland structure and function

JH Widdicombe, JJ Wine - Physiological reviews, 2015 - journals.physiology.org
Submucosal glands contribute to airway surface liquid (ASL), a film that protects all airway
surfaces. Glandular mucus comprises electrolytes, water, the gel-forming mucin MUC5B …

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 …

Airway epithelial repair, regeneration, and remodeling after injury in chronic obstructive pulmonary disease

E Puchelle, JM Zahm, JM Tournier… - Proceedings of the …, 2006 - atsjournals.org
In chronic obstructive pulmonary disease (COPD), exacerbations are generally associated
with several causes, including pollutants, viruses, bacteria that are responsible for an excess …

Role of CFTR in airway disease

JM Pilewski, RA Frizzell - Physiological reviews, 1999 - journals.physiology.org
Pilewski, Joseph M., and Raymond A. Frizzell. Role of CFTR in Airway Disease. Physiol.
Rev. 79, Suppl.: S215–S255, 1999.—Cystic fibrosis (CF) is caused by mutations in the gene …

The porcine lung as a potential model for cystic fibrosis

CS Rogers, WM Abraham… - … of Physiology-Lung …, 2008 - journals.physiology.org
Airway disease currently causes most of the morbidity and mortality in patients with cystic
fibrosis (CF). However, understanding the pathogenesis of CF lung disease and developing …

Characterization of wild-type and ΔF508 cystic fibrosis transmembrane regulator in human respiratory epithelia

SM Kreda, M Mall, A Mengos, L Rochelle… - Molecular biology of …, 2005 - Am Soc Cell Biol
Previous studies in native tissues have produced conflicting data on the localization and
metabolic fate of WT and ΔF508 cystic fibrosis transmembrane regulator (CFTR) in the lung …

Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease

AS Verkman, Y Song… - American Journal of …, 2003 - journals.physiology.org
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) protein, an epithelial chloride channel expressed in the airways, pancreas, testis …

Bicarbonate and chloride secretion in Calu-3 human airway epithelial cells

DC Devor, AK Singh, LC Lambert, A DeLuca… - The Journal of general …, 1999 - rupress.org
Serous cells are the predominant site of cystic fibrosis transmembrane conductance
regulator expression in the airways, and they make a significant contribution to the volume …

Human airway mucin glycosylation: a combinatory of carbohydrate determinants which vary in cystic fibrosis

G Lamblin, S Degroote, JM Perini, P Delmotte… - Glycoconjugate …, 2001 - Springer
Human airway mucins represent a very broad family of polydisperse high molecular mass
glycoproteins, which are part of the airway innate immunity. Apomucins, which correspond to …

Submucosal gland secretions in airways from cystic fibrosis patients have normal [Na+] and pH but elevated viscosity

S Jayaraman, NS Joo, B Reitz… - Proceedings of the …, 2001 - National Acad Sciences
Fluid and macromolecule secretion by submucosal glands in mammalian airways is
believed to be important in normal airway physiology and in the pathophysiology of cystic …