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 …

Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
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 …

[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 …

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 …

CFTR: cystic fibrosis and beyond

MA Mall, D Hartl - 2014 - Eur Respiratory Soc
Cystic fibrosis (CF) remains the most common fatal hereditary lung disease. The discovery of
the cystic fibrosis transmembrane conductance regulator (CFTR) gene 25 years ago set the …

Airway mucus in cystic fibrosis

E Puchelle, O Bajolet, M Abély - Paediatric respiratory reviews, 2002 - Elsevier
Defective expression and function of the cystic fibrosis transmembrane conductance
regulator (CFTR) in cystic fibrosis (CF) airway epithelial cells are associated with airway …

Restoring airway surface liquid in cystic fibrosis

F Ratjen - New England Journal of Medicine, 2006 - Mass Medical Soc
The current pathophysiological model of cystic fibrosis lung disease assumes that defective
expression, trafficking, or function of the cystic fibrosis transmembrane regulator (CFTR) …

Cystic fibrosis: impaired bicarbonate secretion and mucoviscidosis

PM Quinton - The Lancet, 2008 - thelancet.com
For more than 20 years, the abnormally thick mucus (mucoviscidosis) in cystic fibrosis has
been widely shown to be linked to a genetic defect in the cystic fibrosis transmembrane …

New concepts of the pathogenesis of cystic fibrosis lung disease

RC Boucher - European Respiratory Journal, 2004 - Eur Respiratory Soc
Although there has been impressive progress in the elucidation of the genetic and molecular
basis of cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The …

Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator–dependent bicarbonate secretion

MAS Garcia, N Yang, PM Quinton - The Journal of clinical …, 2009 - Am Soc Clin Investig
The mechanisms underlying mucus-associated pathologies in cystic fibrosis (CF) remain
obscure. However, recent studies indicate that CF transmembrane conductance regulator …