[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] Evolutionary adaptations of biofilms infecting cystic fibrosis lungs promote mechanical toughness by adjusting polysaccharide production

K Kovach, M Davis-Fields, Y Irie, K Jain… - npj Biofilms and …, 2017 - nature.com
Biofilms are communities of microbes embedded in a matrix of extracellular polymeric
substances, largely polysaccharides. Multiple types of extracellular polymeric substances …

[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 …

The role of mucus as an invisible cloak to transepithelial drug delivery by nanoparticles

M Garcia-Diaz, D Birch, F Wan, HM Nielsen - Advanced Drug Delivery …, 2018 - Elsevier
Mucosal administration of drugs and drug delivery systems has gained increasing interest.
However, nanoparticles intended to protect and deliver drugs to epithelial surfaces require …

Small-molecule drugs for cystic fibrosis: Where are we now?

O Laselva, L Guerra, S Castellani, M Favia… - Pulmonary …, 2022 - Elsevier
The cystic fibrosis (CF) lung disease is due to the lack/dysfunction of the CF Transmembrane
Conductance Regulator (CFTR), a chloride channel expressed by epithelial cells as the …

Comparison of physicochemical properties of native mucus and reconstituted mucin gels

CE Wagner, M Krupkin, KB Smith-Dupont… - …, 2023 - ACS Publications
Simulating native mucus with model systems such as gels made from reconstituted mucin or
commercially available polymers presents experimental advantages including greater …

CFTR, bicarbonate, and the pathophysiology of cystic fibrosis

D Borowitz - Pediatric pulmonology, 2015 - Wiley Online Library
The gene that encodes for the cystic fibrosis transmembrane regulator protein (CFTR) was
identified in 1989, yet major pathophysiologic questions remain unanswered. There is …

The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis lung disease

PJ Moore, R Tarran - Expert opinion on therapeutic targets, 2018 - Taylor & Francis
Introduction: Cystic fibrosis is an autosomal recessive disorder caused by mutations in the
cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for the CFTR …

Cystic fibrosis-related diabetes and lung disease: an update

BJ Prentice, A Jaffe, S Hameed… - European …, 2021 - Eur Respiratory Soc
The development of cystic fibrosis-related diabetes (CFRD) often leads to poorer outcomes
in patients with cystic fibrosis including increases in pulmonary exacerbations, poorer lung …

Mode of nitric oxide delivery affects antibacterial action

JR Hall, KR Rouillard, DJ Suchyta… - ACS biomaterials …, 2019 - ACS Publications
Nitric oxide (NO) is a broad-spectrum antibacterial agent, making it an attractive alternative
to traditional antibiotics for treating infections. To date, a direct comparison of the …