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 …
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 …
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 …
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 …
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
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) …
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 …
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 …
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 …
transmembrane conductance regulator (CFTR) protein, a plasma membrane protein …
Advances in preclinical in vitro models for the translation of precision medicine for cystic fibrosis
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 …
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 …
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 …
exocrine glands, particularly the lungs, but also the pancreas, liver, kidneys, and intestines …