Role of CFTR in epithelial physiology
Salt and fluid absorption and secretion are two processes that are fundamental to epithelial
function and whole body fluid homeostasis, and as such are tightly regulated in epithelial …
function and whole body fluid homeostasis, and as such are tightly regulated in epithelial …
Airway gland structure and function
JH Widdicombe, JJ Wine - Physiological reviews, 2015 - journals.physiology.org
Submucosal glands contribute to airway surface liquid (ASL), a film that protects all airway
surfaces. Glandular mucus comprises electrolytes, water, the gel-forming mucin MUC5B …
surfaces. Glandular mucus comprises electrolytes, water, the gel-forming mucin MUC5B …
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung
Cystic fibrosis (CF) is a life-shortening disease caused by mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene. Although bacterial lung infection and …
transmembrane conductance regulator (CFTR) gene. Although bacterial lung infection and …
Airway surface liquid pH regulation in airway epithelium current understandings and gaps in knowledge
M Zajac, E Dreano, A Edwards, G Planelles… - International journal of …, 2021 - mdpi.com
Knowledge on the mechanisms of acid and base secretion in airways has progressed
recently. The aim of this review is to summarize the known mechanisms of airway surface …
recently. The aim of this review is to summarize the known mechanisms of airway surface …
Association of TMEM16A chloride channel overexpression with airway goblet cell metaplasia
Key Points• Chloride channels are important for proper hydration of the airway surface.•
TMEM16A protein is an important component of calcium‐activated chloride channels.• …
TMEM16A protein is an important component of calcium‐activated chloride channels.• …
[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 …
encoding CF transmembrane conductance regulator (CFTR), which is a chloride and …
Airway surface liquid acidification initiates host defense abnormalities in Cystic Fibrosis
J Simonin, E Bille, G Crambert, S Noel, E Dreano… - Scientific reports, 2019 - nature.com
Cystic fibrosis (CF) is caused by defective Cystic Fibrosis Transmembrane Conductance
Regulator (CFTR) protein. Morbidity is mainly due to early airway infection. We hypothesized …
Regulator (CFTR) protein. Morbidity is mainly due to early airway infection. We hypothesized …
Dynamic regulation of airway surface liquid pH by TMEM16A and SLC26A4 in cystic fibrosis nasal epithelia with rare mutations
L Delpiano, LW Rodenburg, M Burke… - Proceedings of the …, 2023 - National Acad Sciences
In cystic fibrosis (CF), defects in the CF transmembrane conductance regulator (CFTR)
channel lead to an acidic airway surface liquid (ASL), which compromises innate defence …
channel lead to an acidic airway surface liquid (ASL), which compromises innate defence …
Goblet cell hyperplasia requires high bicarbonate transport to support mucin release
G Gorrieri, P Scudieri, E Caci, M Schiavon, V Tomati… - Scientific reports, 2016 - nature.com
Goblet cell hyperplasia, a feature of asthma and other respiratory diseases, is driven by the
Th-2 cytokines IL-4 and IL-13. In human bronchial epithelial cells, we find that IL-4 induces …
Th-2 cytokines IL-4 and IL-13. In human bronchial epithelial cells, we find that IL-4 induces …
The revolution of personalized pharmacotherapies for cystic fibrosis: what does the future hold?
Introduction Cystic fibrosis (CF), a potentially fatal genetic disease, is caused by loss-of-
function mutations in the gene encoding for the CFTR chloride/bicarbonate channel …
function mutations in the gene encoding for the CFTR chloride/bicarbonate channel …