Journal
JOURNAL OF CYSTIC FIBROSIS
Volume 18, Issue 6, Pages 752-761Publisher
ELSEVIER
DOI: 10.1016/j.jcf.2019.07.005
Keywords
Cystic fibrosis; Endothelial cells; Angiogenesis; Coagulation; Inflammation; Vascular tone; Endothelial barrier
Categories
Funding
- King Baudouin Foundation [2017-J1810150-E008]
- Bettencourt Schueller Foundation
- VIB TechWatch program
- Flemish Government
- Research Foundation Flanders (FWO-Vlaanderen)
- Foundation against Cancer [2016-078]
- Kom op Tegen Kanker (Stand up to Cancer, Flemish Cancer Society)
- ERC Proof of Concept (ERC) [713758]
- ERC Advanced Research Grant (EU-ERC) [743074]
- clinical research fund University Hospitals Leuven
- European Research Council (ERC) [713758, 743074] Funding Source: European Research Council (ERC)
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Cystic fibrosis (CF) is an autosomal recessive disease caused by the loss of function of the cystic fibrosis transmembrane conductance regulator (CFTR) protein which primarily acts as a chloride channel. CFTR has mainly been studied in epithelial cells although it is also functional and expressed in other cell types including endothelial cells. The present review summarizes current knowledge on the role of the endothelium in CF. More specifically, this review highlights the role of endothelial cells in CF in acting as a semipermeable barrier, as a key regulator of angiogenesis, coagulation, the vascular tone and the inflammatory responses. It could contribute to different aspects of the disease including cardiovascular symptoms, excessive blood vessel formation, pulmonary and portal hypertension and CF-related diabetes. Despite the important role of vascular endothelium in many biological processes, it has largely been under investigated in CF. (C) 2019 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
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