[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 …

Dysfunctional inflammation in cystic fibrosis airways: from mechanisms to novel therapeutic approaches

A Ghigo, G Prono, E Riccardi, V De Rose - International Journal of …, 2021 - mdpi.com
Cystic fibrosis (CF) is an inherited disorder caused by mutations in the gene encoding for the
cystic fibrosis transmembrane conductance regulator (CFTR) protein, an ATP-gated chloride …

Proton leak and CFTR in regulation of Golgi pH in respiratory epithelial cells

G Chandy, M Grabe, HPH Moore… - American Journal of …, 2001 - journals.physiology.org
Work addressing whether cystic fibrosis transmembrane conductance regulator (CFTR)
plays a role in regulating organelle pH has remained inconclusive. We engineered a pH …

Cystic fibrosis transmembrane conductance regulator-associated ATP and adenosine 3′-phosphate 5′-phosphosulfate channels in endoplasmic reticulum and …

EA Pasyk, JK Foskett - Journal of Biological Chemistry, 1997 - ASBMB
Cystic fibrosis (CF) is characterized by abnormal regulation of epithelial ion and fluid
transport due to mutations in the CF transmembrane conductance regulator (CFTR), an …

Macrophage dysfunction in cystic fibrosis: Nature or nurture?

KB Turton, RJ Ingram… - Journal of Leucocyte …, 2021 - academic.oup.com
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) affect the
homeostasis of chloride flux by epithelial cells. This has deleterious consequences …

CFTR and calcium-activated chloride channels in primary cultures of human airway gland cells of serous or mucous phenotype

H Fischer, B Illek, L Sachs… - … of Physiology-Lung …, 2010 - journals.physiology.org
Using cell culture models, we have investigated the relative importance of cystic fibrosis
transmembrane conductance regulator (CFTR) and calcium-activated chloride channels …

Extracellular zinc and ATP restore chloride secretion across cystic fibrosis airway epithelia by triggering calcium entry

A Zsembery, JA Fortenberry, L Liang, Z Bebok… - Journal of Biological …, 2004 - ASBMB
Cystic fibrosis (CF) is caused by defective cyclic AMP-dependent cystic fibrosis
transmembrane conductance regulator Cl-channels. Thus, CF epithelia fail to transport Cl …

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] Update on Calcium signaling in cystic fibrosis lung disease

A Rimessi, VAM Vitto, S Patergnani… - Frontiers in …, 2021 - frontiersin.org
Cystic fibrosis (CF) is an autosomal recessive disorder characterized by mutations in the
cystic fibrosis transmembrane conductance regulator gene, which causes multifunctional …

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 …