[HTML][HTML] Role of CFTR in epithelial physiology

V Saint-Criq, MA Gray - Cellular and Molecular Life Sciences, 2017 - Springer
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 …

[HTML][HTML] 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 …

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung

AA Pezzulo, XX Tang, MJ Hoegger, MH Abou Alaiwa… - Nature, 2012 - nature.com
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 …

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 …

Association of TMEM16A chloride channel overexpression with airway goblet cell metaplasia

P Scudieri, E Caci, S Bruno, L Ferrera… - The Journal of …, 2012 - Wiley Online Library
Key Points• Chloride channels are important for proper hydration of the airway surface.•
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 …

[HTML][HTML] 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 …

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 …

[HTML][HTML] 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 …

The revolution of personalized pharmacotherapies for cystic fibrosis: what does the future hold?

KE Oliver, MS Carlon, N Pedemonte… - Expert Opinion on …, 2023 - Taylor & Francis
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 …