[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 …
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 …
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 …
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 …
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 …
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
Using cell culture models, we have investigated the relative importance of cystic fibrosis
transmembrane conductance regulator (CFTR) and calcium-activated chloride channels …
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 …
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 …
obscure. However, recent studies indicate that CF transmembrane conductance regulator …
[HTML][HTML] Update on Calcium signaling in cystic fibrosis lung disease
Cystic fibrosis (CF) is an autosomal recessive disorder characterized by mutations in the
cystic fibrosis transmembrane conductance regulator gene, which causes multifunctional …
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 …
Rev. 79, Suppl.: S215–S255, 1999.—Cystic fibrosis (CF) is caused by mutations in the gene …