Title |
Aberrant GSH reductase and NOX activities concur with defective CFTR to pro-oxidative imbalance in cystic fibrosis airways
|
---|---|
Published in |
Journal of Bioenergetics and Biomembranes, March 2018
|
DOI | 10.1007/s10863-018-9748-x |
Pubmed ID | |
Authors |
L. de Bari, M. Favia, A. Bobba, R. Lassandro, L. Guerra, A. Atlante |
Abstract |
Cystic fibrosis (CF) is associated to impaired Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) channel also causing decreased glutathione (GSH) secretion, defective airway bacterial clearance and inflammation. Here we checked the main ROS-producing and ROS-scavenging enzymes as potential additional factors involved in CF pathogenesis. We found that CFBE41o-cells, expressing F508del CFTR, have increased NADPH oxidase (NOX) activity and expression level, mainly responsible of the increased ROS production, and decreased glutathione reductase (GR) activity, not dependent on GR protein level decrease. Furthermore, defective CFTR proved to cause both extracellular and intracellular GSH level decrease, probably by reducing the amount of extracellular GSH-derived cysteine required for cytosolic GSH synthesis. Importantly, we provide evidence that defective CFTR and NOX/GR activity imbalance both contribute to NADPH and GSH level decrease and ROS overproduction in CF cells. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 2 | 100% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 2 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 22 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Researcher | 4 | 18% |
Student > Doctoral Student | 3 | 14% |
Student > Bachelor | 3 | 14% |
Student > Ph. D. Student | 3 | 14% |
Student > Master | 3 | 14% |
Other | 2 | 9% |
Unknown | 4 | 18% |
Readers by discipline | Count | As % |
---|---|---|
Biochemistry, Genetics and Molecular Biology | 5 | 23% |
Immunology and Microbiology | 4 | 18% |
Medicine and Dentistry | 3 | 14% |
Pharmacology, Toxicology and Pharmaceutical Science | 1 | 5% |
Computer Science | 1 | 5% |
Other | 3 | 14% |
Unknown | 5 | 23% |