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J Cyst Fibros. 2019 Aug 7. pii: S1569-1993(19)30826-4. doi: 10.1016/j.jcf.2019.07.005. [Epub ahead of print]

The role of endothelial cells in cystic fibrosis.

Author information

1
Department of Development and Regeneration, Faculty of Medicine, KU Leuven, Leuven, Belgium; Laboratory of Angiogenesis and Vascular Metabolism, Center for Cancer Biology, VIB, Leuven, Belgium; Laboratory of Angiogenesis and Vascular Metabolism, Department of Oncology and Leuven Cancer Institute (LKI), KU Leuven, Leuven, Belgium.
2
Laboratory of Angiogenesis and Vascular Metabolism, Center for Cancer Biology, VIB, Leuven, Belgium; Laboratory of Angiogenesis and Vascular Metabolism, Department of Oncology and Leuven Cancer Institute (LKI), KU Leuven, Leuven, Belgium.
3
Department of Development and Regeneration, Faculty of Medicine, KU Leuven, Leuven, Belgium; Department of Paediatrics and center of metabolic diseases, University Hospitals Leuven, Leuven, Belgium; Department of Development and Regeneration, University Hospitals Leuven, Leuven, Belgium. Electronic address: peter.witters@uzleuven.be.

Abstract

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.

KEYWORDS:

Angiogenesis; Coagulation; Cystic fibrosis; Endothelial barrier; Endothelial cells; Inflammation; Vascular tone

PMID:
31401006
DOI:
10.1016/j.jcf.2019.07.005

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