Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond

MA Mall, GJ Criner, M Miravitlles… - European …, 2023 - Eur Respiratory Soc
The cystic fibrosis transmembrane conductance regulator (CFTR) is a crucial ion channel for
transport of chloride and bicarbonate anions. Functional roles of CFTR have been identified …

Vaporized E-cigarette liquids induce ion transport dysfunction in airway epithelia

VY Lin, MD Fain, PL Jackson, TF Berryhill… - American journal of …, 2019 - atsjournals.org
Cigarette smoking is associated with chronic obstructive pulmonary disease and chronic
bronchitis. Acquired ion transport abnormalities, including cystic fibrosis transmembrane …

Ivacaftor reverses airway mucus abnormalities in a rat model harboring a humanized G551D-CFTR

SE Birket, JM Davis, CM Fernandez-Petty… - American journal of …, 2020 - atsjournals.org
Rationale: Animal models have been highly informative for understanding the
characteristics, onset, and progression of cystic fibrosis (CF) lung disease. In particular, the …

[HTML][HTML] A novel G542X CFTR rat model of cystic fibrosis is sensitive to nonsense mediated decay

J Sharma, J Abbott, L Klaskala, G Zhao… - Frontiers in …, 2020 - frontiersin.org
Nonsense mutations that lead to the insertion of a premature termination codon (PTC) in the
cystic fibrosis transmembrane conductance regulator (CFTR) transcript affect 11% of …

Evaluation of secondhand smoke effects on CFTR function in vivo

LW Rasmussen, D Stanford, K Patel, SV Raju - Respiratory Research, 2020 - Springer
Background Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion
channel expressed on the mucosal surface of epithelial cells lining several tissues including …

Pulmonary arterial hypertension and CFTR: the paradox of going forward by tacking sideways!

T Kotsimbos, D Kaye, D Keating - European Respiratory …, 2021 - Eur Respiratory Soc
The understanding of pulmonary arterial hypertension (PAH) was turned on its head just
over 20 years ago with the recognition that there were families with a specific genetic …

Gene Environment Interactions

S Shanthikumar - Hodson and Geddes' Cystic Fibrosis, 2023 - taylorfrancis.com
People with cystic fibrosis express significant variability in their clinical phenotype, despite
the disease being caused by mutations in a single gene. To inform efforts to reduce or …