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 …

Organoid technology and its role for theratyping applications in cystic fibrosis

J Conti, C Sorio, P Melotti - Children, 2022 - mdpi.com
Cystic fibrosis (CF) is a autosomal recessive, multisystemic disease caused by different
mutations in the CFTR gene encoding CF transmembrane conductance regulator. Although …

Choice of differentiation media significantly impacts cell lineage and response to CFTR modulators in fully differentiated primary cultures of cystic fibrosis human …

V Saint-Criq, L Delpiano, J Casement, JC Onuora… - Cells, 2020 - mdpi.com
In vitro cultures of primary human airway epithelial cells (hAECs) grown at air–liquid
interface have become a valuable tool to study airway biology under normal and pathologic …

CFTR modulators restore acidification of autophago-lysosomes and bacterial clearance in cystic fibrosis macrophages

A Badr, M Eltobgy, K Krause, K Hamilton… - Frontiers in Cellular …, 2022 - frontiersin.org
Cystic fibrosis (CF) human and mouse macrophages are defective in their ability to clear
bacteria such as Burkholderia cenocepacia. The autophagy process in CF (F508del) …

[HTML][HTML] Rescue of chloride and bicarbonate transport by elexacaftor-ivacaftor-tezacaftor in organoid-derived CF intestinal and cholangiocyte monolayers

MJC Bijvelds, FJM Roos, KF Meijsen, HP Roest… - Journal of cystic …, 2022 - Elsevier
Background In cystic fibrosis (CF), loss of CF transmembrane conductance regulator (CFTR)-
dependent bicarbonate secretion precipitates the accumulation of viscous mucus in the …

[HTML][HTML] From membrane to nucleus: A three-wave hypothesis of cAMP signaling

A Pizzoni, X Zhang, DL Altschuler - Journal of Biological Chemistry, 2023 - Elsevier
For many decades, our understanding of G protein-coupled receptor (GPCR) activity and
cyclic AMP (cAMP) signaling was limited exclusively to the plasma membrane. However, a …

Modulator combination improves in vitro the microrheological properties of the airway surface liquid of cystic fibrosis airway epithelia

A Ludovico, O Moran, D Baroni - International Journal of Molecular …, 2022 - mdpi.com
Cystic fibrosis (CF) is a genetic disease caused by mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) protein, a plasma membrane protein …

Advances in preclinical in vitro models for the translation of precision medicine for cystic fibrosis

IAL Silva, O Laselva, M Lopes-Pacheco - Journal of Personalized …, 2022 - mdpi.com
The development of preclinical in vitro models has provided significant progress to the
studies of cystic fibrosis (CF), a frequently fatal monogenic disease caused by mutations in …

Bicarbonate transport in cystic fibrosis and pancreatitis

D Angyal, MJC Bijvelds, MJ Bruno, MP Peppelenbosch… - Cells, 2021 - mdpi.com
CFTR, the cystic fibrosis (CF) gene-encoded epithelial anion channel, has a prominent role
in driving chloride, bicarbonate and fluid secretion in the ductal cells of the exocrine …

The application of bicarbonate recovers the chemical-physical properties of airway surface liquid in cystic fibrosis epithelia models

L Ferrera, V Capurro, L Delpiano, A Gianotti, O Moran - Biology, 2021 - mdpi.com
Simple Summary Cystic fibrosis (CF) is a multi-organ disease that affects the epithelia and
exocrine glands, particularly the lungs, but also the pancreas, liver, kidneys, and intestines …