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 …

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 …

[HTML][HTML] Bicarbonate in cystic fibrosis

K Kunzelmann, R Schreiber, HB Hadorn - Journal of Cystic Fibrosis, 2017 - Elsevier
Background Cystic fibrosis (CF, mucoviscidosis) is caused by mutations in the gene
encoding CF transmembrane conductance regulator (CFTR), which is a chloride and …

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 …

CFTR, bicarbonate, and the pathophysiology of cystic fibrosis

D Borowitz - Pediatric pulmonology, 2015 - Wiley Online Library
The gene that encodes for the cystic fibrosis transmembrane regulator protein (CFTR) was
identified in 1989, yet major pathophysiologic questions remain unanswered. There is …

Cellular localization of the cystic fibrosis transmembrane conductance regulator in mouse intestinal tract

N Ameen, J Alexis, P Salas - Histochemistry and cell biology, 2000 - Springer
The cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP and cGMP-
regulated chloride channel critical to the regulation of intestinal fluid, chloride, and …

[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 …

Cystic fibrosis: pathophysiology of lung disease

C Bergeron, AM Cantin - Seminars in respiratory and critical …, 2019 - thieme-connect.com
Cystic fibrosis (CF) is a common, life-threatening, multisystemic, autosomal recessive
disorder. In the last few years, giant steps have been made with regard to the understanding …

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 …

[HTML][HTML] CFTR protein: not just a chloride channel?

LS Hanssens, J Duchateau, GJ Casimir - Cells, 2021 - mdpi.com
Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in a gene encoding
a protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). The CFTR …