[HTML][HTML] Cystic fibrosis: an inherited disease affecting mucin-producing organs
C Ehre, C Ridley, DJ Thornton - The international journal of biochemistry & …, 2014 - Elsevier
Our current understanding of cystic fibrosis (CF) has revealed that the biophysical properties
of mucus play a considerable role in the pathogenesis of the disease in view of the fact that …
of mucus play a considerable role in the pathogenesis of the disease in view of the fact that …
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, mucins, and cystic fibrosis
Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …
Bicarbonate and functional CFTR channel are required for proper mucin secretion and link cystic fibrosis with its mucus phenotype
JK Gustafsson, A Ermund, D Ambort… - Journal of Experimental …, 2012 - rupress.org
Cystic fibrosis (CF) is caused by a nonfunctional chloride and bicarbonate ion channel (CF
transmembrane regulator [CFTR]), but the link to the phenomenon of stagnant mucus is not …
transmembrane regulator [CFTR]), but the link to the phenomenon of stagnant mucus is not …
Constitutive mucin secretion linked to CFTR expression
R Kuver, N Ramesh, S Lau, C Savard, SP Lee… - Biochemical and …, 1994 - Elsevier
Mucus plugging is a hallmark of cystic fibrosis, but the link between the defective gene
product, the cystic fibrosis transmembrane conductance regulator, and the abnormal mucus …
product, the cystic fibrosis transmembrane conductance regulator, and the abnormal mucus …
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 …
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 …
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 …
The cystic fibrosis intestine
RC De Lisle, D Borowitz - Cold Spring …, 2013 - perspectivesinmedicine.cshlp.org
The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis
transmembrane regulator protein (CFTR). The majority of people with CF have a limited life …
transmembrane regulator protein (CFTR). The majority of people with CF have a limited life …