[HTML][HTML] Increased expression of ATP12A proton pump in cystic fibrosis airways

P Scudieri, I Musante, E Caci, A Venturini, P Morelli… - Jci Insight, 2018 - ncbi.nlm.nih.gov
Proton secretion mediated by ATP12A protein on the surface of the airway epithelium may
contribute to cystic fibrosis (CF) lung disease by favoring bacterial infection and airway …

Hyperglycaemia and Pseudomonas aeruginosa acidify cystic fibrosis airway surface liquid by elevating epithelial monocarboxylate transporter 2 dependent lactate-H+ …

JP Garnett, KK Kalsi, M Sobotta, J Bearham, G Carr… - Scientific reports, 2016 - nature.com
The cystic fibrosis (CF) airway surface liquid (ASL) provides a nutrient rich environment for
bacterial growth including elevated glucose, which together with defective bacterial killing …

Discovery of icenticaftor (QBW251), a cystic fibrosis transmembrane conductance regulator potentiator with clinical efficacy in cystic fibrosis and chronic obstructive …

DL Grand, M Gosling, U Baettig, P Bahra… - Journal of Medicinal …, 2021 - ACS Publications
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) ion channel
are established as the primary causative factor in the devastating lung disease cystic fibrosis …

Breath concentration of acetic acid vapour is elevated in patients with cystic fibrosis

D Smith, K Sovová, K Dryahina… - Journal of breath …, 2016 - iopscience.iop.org
A study has been carried out of the volatile organic compounds present in the exhaled
breath of 58 cystic fibrosis (CF) patients. An important observation is that the acetic acid …

Inhibition of protease–epithelial sodium channel signaling improves mucociliary function in cystic fibrosis airways

JA Reihill, B Walker, RA Hamilton… - American journal of …, 2016 - atsjournals.org
Rationale: In cystic fibrosis (CF) a reduction in airway surface liquid (ASL) height
compromises mucociliary clearance, favoring mucus plugging and chronic bacterial …

Modulation of engineered nanomaterial interactions with organ barriers for enhanced drug transport

V Lenders, X Koutsoumpou, P Phan… - Chemical Society …, 2023 - pubs.rsc.org
The biomedical use of nanoparticles (NPs) has been the focus of intense research for over a
decade. As most NPs are explored as carriers to alter the biodistribution, pharmacokinetics …

A SPLUNC1 peptidomimetic inhibits Orai1 and reduces inflammation in a murine allergic asthma model

JA Wrennall, S Ahmad, EN Worthington… - American Journal of …, 2022 - atsjournals.org
Orai1 is a plasma membrane Ca2+ channel that mediates store-operated Ca2+ entry
(SOCE) and regulates inflammation. Short palate lung and nasal epithelial clone 1 …

Recombinant acid ceramidase reduces inflammation and infection in cystic fibrosis

AI Gardner, IJ Haq, AJ Simpson, KA Becker… - American Journal of …, 2020 - atsjournals.org
Rationale: In cystic fibrosis the major cause of morbidity and mortality is lung disease
characterized by inflammation and infection. The influence of sphingolipid metabolism is …

[HTML][HTML] Airway surface hyperviscosity and defective mucociliary transport by IL-17/TNF-α are corrected by β-adrenergic stimulus

D Guidone, M Buccirossi, P Scudieri, M Genovese… - JCI insight, 2022 - ncbi.nlm.nih.gov
The fluid covering the surface of airway epithelia represents a first barrier against
pathogens. The chemical and physical properties of the airway surface fluid are controlled …

Engineered cationic antimicrobial peptide (eCAP) prevents Pseudomonas aeruginosa biofilm growth on airway epithelial cells

LP Lashua, JA Melvin, B Deslouches… - Journal of …, 2016 - academic.oup.com
Objectives Chronic infections with the opportunistic pathogen Pseudomonas aeruginosa are
responsible for the majority of the morbidity and mortality in patients with cystic fibrosis (CF) …