Article ; Online: Cystic Fibrosis and Oxidative Stress: The Role of CFTR.
Molecules (Basel, Switzerland)
2022 Volume 27, Issue 16
Abstract: There is substantial evidence in the literature that patients with cystic fibrosis (CF) have higher oxidative stress than patients with other diseases or healthy subjects. This results in an increase in reactive oxygen species (ROS) and in a deficit of ... ...
| Abstract | There is substantial evidence in the literature that patients with cystic fibrosis (CF) have higher oxidative stress than patients with other diseases or healthy subjects. This results in an increase in reactive oxygen species (ROS) and in a deficit of antioxidant molecules and plays a fundamental role in the progression of chronic lung damage. Although it is known that recurrent infection-inflammation cycles in CF patients generate a highly oxidative environment, numerous clinical and preclinical studies suggest that the airways of a patient with CF present an inherently abnormal proinflammatory milieu due to elevated oxidative stress and abnormal lipid metabolism even before they become infected. This could be directly related to cystic fibrosis transmembrane conductance regulator (CFTR) deficiency, which appears to produce a redox imbalance in epithelial cells and extracellular fluids. This review aims to summarize the main mechanism by which CFTR deficiency is intrinsically responsible for the proinflammatory environment that characterizes the lung of a patient with CF. |
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| MeSH term(s) | Cystic Fibrosis/genetics ; Cystic Fibrosis/metabolism ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/metabolism ; Humans ; Lung/metabolism ; Oxidative Stress ; Reactive Oxygen Species/metabolism | |||||
| Chemical Substances | CFTR protein, human ; Reactive Oxygen Species ; Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6) | |||||
| Language | English | |||||
| Publishing date | 2022-08-21 | |||||
| Publishing country | Switzerland | |||||
| Document type | Journal Article ; Review | |||||
| ZDB-ID | 1413402-0 | |||||
| ISSN | 1420-3049 ; 1431-5165 ; 1420-3049 | |||||
| ISSN (online) | 1420-3049 | |||||
| ISSN | 1431-5165 ; 1420-3049 | |||||
| DOI | 10.3390/molecules27165324 | |||||
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| Database | MEDical Literature Analysis and Retrieval System OnLINE |
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