Article ; Online: Platelets: inflammatory effector cells in the conflagration of cystic fibrosis lung disease.
The Journal of clinical investigation
2020 Volume 130, Issue 4, Page(s) 1632–1634
Abstract: Cystic fibrosis (CF) is a multisystem disorder, but progressive inflammatory lung disease causes the greatest burden of morbidity and death. Recent translational and mechanistic studies of samples from patients, and observations in animal models, ... ...
Abstract | Cystic fibrosis (CF) is a multisystem disorder, but progressive inflammatory lung disease causes the greatest burden of morbidity and death. Recent translational and mechanistic studies of samples from patients, and observations in animal models, indicate that platelets may drive lung injury and contribute to dysregulated host defense in CF lung disease. In this issue of the JCI, Ortiz-Muñoz and Yu et al. explored the role that the cystic fibrosis transmembrane conductance regulator (CFTR) plays in platelet-related inflammation. The authors used mouse and human model systems to show that CFTR dysfunction in platelets increased calcium entry though the transient receptor potential cation channel 6 (TRPC6), causing hyperactivation and consequent experimental lung inflammation. The study persuasively suggests that platelets are critical thromboinflammatory effector cells in CF lung disease. In the context of platelet-related organ injury seen in a variety of other diseases and syndromes, platelets may also contribute to nonpulmonary manifestations and comorbidities of CF. |
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MeSH term(s) | Animals ; Blood Platelets/metabolism ; Cystic Fibrosis ; Cystic Fibrosis Transmembrane Conductance Regulator ; Humans ; Ion Transport ; Lung/metabolism ; Mice |
Chemical Substances | Cystic Fibrosis Transmembrane Conductance Regulator (126880-72-6) |
Language | English |
Publishing date | 2020-03-16 |
Publishing country | United States |
Document type | Journal Article ; Research Support, N.I.H., Extramural ; Comment |
ZDB-ID | 3067-3 |
ISSN | 1558-8238 ; 0021-9738 |
ISSN (online) | 1558-8238 |
ISSN | 0021-9738 |
DOI | 10.1172/JCI135949 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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