Pulmonary Ionocytes Regulate Airway Surface Liquid pH in Primary Human Bronchial Epithelial Cells

X Luan, N Henao Romero, VA Campanucci… - … Journal of Respiratory …, 2024 - atsjournals.org
… to ASL formation but regulate ASL pH by secreting bicarbonate via CFTR-linked Cl-/bicarbonate …
cystic fibrosis transmembrane conductance regulator (CFTR) anion channel at a higher …

Mucins and CFTR: their close relationship

K Okuda, KM Shaffer, C Ehre - International journal of molecular sciences, 2022 - mdpi.com
… cystic fibrosis transmembrane conductance regulator (CFTR) … : (1) reduction in airway surface
liquid (ASL) volume, mucus … expansion via the exchange of one Ca 2+ for two Na + ions […

A single-cell atlas of large and small airways at birth in a porcine model of cystic fibrosis

AL Thurman, X Li, R Villacreses, W Yu… - … journal of respiratory …, 2022 - atsjournals.org
… of CFTR-rich ionocytes in small airways, we expected CFTRconductance of small airway
epithelia at the air–liquid … Assuming uniform distribution of ionocytes on the airway surface, we …

[HTML][HTML] Accumulation and persistence of ivacaftor in airway epithelia with prolonged treatment

TNG Lee, DM Cholon, NL Quinney, M Gentzsch… - Journal of Cystic …, 2020 - Elsevier
… the cystic fibrosis transmembrane conductance regulator (CFTR), a … for Ca 2+ channel activity
was assessed as an internal control. … Blots were probed with mouse monoclonal anti-CFTR

The short-term rinsing of airways by N-acetylcysteine helps expectoration: The mechanism of sodium and chloride transport

I Hołyńska-Iwan, I Dziembowska… - Advances in Hygiene …, 2020 - phmd.pl
… Both changes in airway surface liquid (ASL) and ciliary activity are regulated by epithelial …
-potassium pump, chloride ion secretion through cystic fibrosis conductance regulator (CFTR), …

Strategies for cystic fibrosis transmembrane conductance regulator inhibition: from molecular mechanisms to treatment for secretory diarrhoeas

HR de Jonge, MC Ardelean, MJC Bijvelds… - FEBS …, 2020 - Wiley Online Library
… - and Ca 2+ -regulated local Cl − and fluid secretion serving to … compounds on anion
conductance in YFP-CFTR-expressing … Moreover, testing CFTR inhibitors in mouse models of SD [[…

CFTR dysfunction leads to defective bacterial eradication on cystic fibrosis airways

M Wu, JH Chen - Frontiers in Physiology, 2024 - frontiersin.org
… salt, low volume, airway surface liquid acidosis and … CFTR-mediated Clconductance may
hinder the apical Na + absorption, so that NaCl may be retained in the airway surface liquid (…

Inhibition of Na+/H+ exchanger isoform 3 improves gut fluidity and alkalinity in cystic fibrosis transmembrane conductance regulator‐deficient and F508del mutant …

Q Tan, G di Stefano, X Tan, X Renjie… - British Journal of …, 2021 - Wiley Online Library
… ability to increase luminal fluidity and alkalinity in cystic fibrosis transmembrane conductance
regulator (CFTR) null (cftr −/− ) and F508del mutant (F508del mut/mut ) murine intestine. …

[HTML][HTML] A glycopolymer improves vascoelasticity and mucociliary transport of abnormal cystic fibrosis mucus

CM Fernandez-Petty, GW Hughes, HL Bowers… - Jci Insight, 2019 - ncbi.nlm.nih.gov
… CF transmembrane regulator–mediated (CFTR-mediated) ion … interacts with mucins in a
Ca 2+ -dependent manner to … based on mean airway surface liquid (ASL) volume of the 0.33 …

Pharmacological modulation of mitochondrial calcium uniporter controls lung inflammation in cystic fibrosis

A Rimessi, C Pozzato, L Carparelli, A Rossi… - Science …, 2020 - science.org
… the CF transmembrane conductance regulator (CFTR) protein (… transport, reducing water
content in the airway surface liquid. … key role in mediating interorganelle Ca 2+ transfer from the …