The role of endothelial cells in cystic fibrosis.
Angiogenesis
Coagulation
Cystic fibrosis
Endothelial barrier
Endothelial cells
Inflammation
Vascular tone
Journal
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
ISSN: 1873-5010
Titre abrégé: J Cyst Fibros
Pays: Netherlands
ID NLM: 101128966
Informations de publication
Date de publication:
11 2019
11 2019
Historique:
received:
24
04
2019
revised:
18
06
2019
accepted:
23
07
2019
pubmed:
12
8
2019
medline:
11
11
2020
entrez:
12
8
2019
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 31401006
pii: S1569-1993(19)30826-4
doi: 10.1016/j.jcf.2019.07.005
pii:
doi:
Substances chimiques
CFTR protein, human
0
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
752-761Informations de copyright
Copyright © 2019. Published by Elsevier B.V.