[HTML][HTML] The Big Impact of Small Airway pH

K Okuda, SH Randell, SE Birket - American Journal of Respiratory …, 2021 - atsjournals.org
Cystic fibrosis (CF) lung disease, caused by abnormal ion transport because of deficient
CFTR (cystic fibrosis transmembrane conductance regulator) function, is characterized by a …

[HTML][HTML] Macrophage dysfunction in cystic fibrosis: a therapeutic target to enhance self-immunity

TL Bonfield - American Journal of Respiratory and Critical Care …, 2015 - atsjournals.org
The primary defect in cystic fibrosis (CF) is a result of mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene, which results in altered sodium and …

[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 …

Cystic fibrosis: a disease of vulnerability to airway surface dehydration

RC Boucher - Trends in molecular medicine, 2007 - cell.com
Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway
secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the …

Early pulmonary inflammation and lung damage in children with cystic fibrosis

A Schultz, S Stick - Respirology, 2015 - Wiley Online Library
Individuals with cystic fibrosis (CF) suffer progressive airway inflammation, infection and
lung damage. Airway inflammation and infection are present from early in life, often before …

[HTML][HTML] Defective Fluid Secretion from Submucosal Glands of Nasal Turbinates from CFTR-/- and CFTRΔF508/ΔF508 Pigs

HJ Cho, NS Joo, JJ Wine - PloS one, 2011 - journals.plos.org
Background Cystic fibrosis (CF), caused by reduced CFTR function, includes severe
sinonasal disease which may predispose to lung disease. Newly developed CF pigs provide …

New models of the tracheal airway define the glandular contribution to airway surface fluid and electrolyte composition

X Wang, Y Zhang, A Amberson… - American journal of …, 2001 - atsjournals.org
Antibacterial defenses in the airway are dependent on multifactorial influences that
determine the composition of both fluid and/or electrolytes at the surface of the airway and …

[HTML][HTML] Chloride transport and inflammation in cystic fibrosis airways

G Varelogianni - 2011 - diva-portal.org
Cystic fibrosis (CF) is one of the most common lethal, autosomal recessive inherited
diseases among Caucasians. It is caused by a mutation in the cystic fibrosis transmembrane …

[PDF][PDF] Mechanisms of acid and base secretion: implications for airway host defense in cystic fibrosis

VS Shah - 2017 - iro.uiowa.edu
The airway surface contains a number of important defense mechanisms to protect against
infection. Antimicrobials found in the thin layer of fluid lining the airways, the airway surface …

Extracellular pH and lung infections in cystic fibrosis

MM Massip-Copiz, TA Santa-Coloma - European journal of cell biology, 2018 - Elsevier
Cystic fibrosis (CF) is an autosomal recessive disease caused by CFTR mutations. It is
characterized by high NaCl concentration in sweat and the production of a thick and sticky …