Stem cell-based Lung-on-Chips: The best of both worlds?

JC Nawroth, R Barrile, D Conegliano, S van Riet… - Advanced drug delivery …, 2019 - Elsevier
Pathologies of the respiratory system such as lung infections, chronic inflammatory lung
diseases, and lung cancer are among the leading causes of morbidity and mortality, killing …

Toward gene therapy for cystic fibrosis using a lentivirus pseudotyped with Sendai virus envelopes

K Mitomo, U Griesenbach, M Inoue, L Somerton… - Molecular therapy, 2010 - cell.com
Gene therapy for cystic fibrosis (CF) is making encouraging progress into clinical trials.
However, further improvements in transduction efficiency are desired. To develop a novel …

The antioxidant role of thiocyanate in the pathogenesis of cystic fibrosis and other inflammation-related diseases

Y Xu, S Szép, Z Lu - … of the National Academy of Sciences, 2009 - National Acad Sciences
Cystic fibrosis (CF) is a pleiotropic disease, originating from mutations in the CF
transmembrane conductance regulator (CFTR). Lung injuries inflicted by recurring infection …

E3KARP mediates the association of ezrin and protein kinase A with the cystic fibrosis transmembrane conductance regulator in airway cells

F Sun, MJ Hug, CM Lewarchik, CHC Yun… - Journal of Biological …, 2000 - ASBMB
Although it is generally recognized that cystic fibrosis transmembrane conductance regulator
(CFTR) contains a PSD-95/Disc-large/ZO-1 (PDZ)-binding motif at its COOH terminus, the …

Rethinking cystic fibrosis pathology: the critical role of abnormal reduced glutathione (GSH) transport caused by CFTR mutation

VM Hudson - Free radical biology and medicine, 2001 - Elsevier
Though the cause of cystic fibrosis (CF) pathology is understood to be the mutation of the
CFTR protein, it has been difficult to trace the exact mechanisms by which the pathology …

Interleukin-13 induces a hypersecretory ion transport phenotype in human bronchial epithelial cells

H Danahay, H Atherton, G Jones… - American Journal of …, 2002 - journals.physiology.org
Interleukin (IL)-13 has been associated with asthma, allergic rhinitis, and chronic sinusitis,
all conditions where an imbalance in epithelial fluid secretion and absorption could impact …

Localization of cystic fibrosis transmembrane conductance regulator to lipid rafts of epithelial cells is required for Pseudomonas aeruginosa-induced cellular activation

MP Kowalski, GB Pier - The Journal of Immunology, 2004 - journals.aai.org
The cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein is an
epithelial cell receptor for the outer core oligosaccharide of the Pseudomonas aeruginosa …

Phenylglycine and sulfonamide correctors of defective ΔF508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating

N Pedemonte, ND Sonawane, A Taddei, J Hu… - Molecular …, 2005 - ASPET
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) chloride
channel cause cystic fibrosis. The ΔF508 mutation produces defects in channel gating and …

[HTML][HTML] Chloride channelopathies

R Planells-Cases, TJ Jentsch - … et Biophysica Acta (BBA)-Molecular Basis …, 2009 - Elsevier
Channelopathies, defined as diseases that are caused by mutations in genes encoding ion
channels, are associated with a wide variety of symptoms. Impaired chloride transport can …

Cystic fibrosis transmembrane conductance regulator: structure and function of an epithelial chloride channel

MH Akabas - Journal of Biological Chemistry, 2000 - ASBMB
The cystic fibrosis transmembrane conductance regulator (CFTR) 1 forms a Cl channel that
is an essential component of epithelial Cl transport systems in many organs, including the …