Deregulation of Notch1 pathway and circulating endothelial progenitor cell (EPC) number in patients with bicuspid aortic valve with and without ascending aorta aneurysm

Abstract Bicuspid aortic valve (BAV) is frequently associated with the development of ascending aortic aneurysm, even if the underlying mechanisms remain to be clarified. Here, we investigated if a deregulation of Notch1 signaling pathway and endothelial progenitor cells (EPCs) number is associated...

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Autores principales: Carmela R. Balistreri, Floriana Crapanzano, Leonardo Schirone, Alberto Allegra, Calogera Pisano, Giovanni Ruvolo, Maurizio Forte, Ernesto Greco, Elena Cavarretta, Antonino G. M. Marullo, Sebastiano Sciarretta, Giacomo Frati
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Publicado: Nature Portfolio 2018
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Acceso en línea:https://doaj.org/article/cb1af62bbb1a40608db4adefa71c4b88
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spelling oai:doaj.org-article:cb1af62bbb1a40608db4adefa71c4b882021-12-02T15:07:48ZDeregulation of Notch1 pathway and circulating endothelial progenitor cell (EPC) number in patients with bicuspid aortic valve with and without ascending aorta aneurysm10.1038/s41598-018-32170-22045-2322https://doaj.org/article/cb1af62bbb1a40608db4adefa71c4b882018-09-01T00:00:00Zhttps://doi.org/10.1038/s41598-018-32170-2https://doaj.org/toc/2045-2322Abstract Bicuspid aortic valve (BAV) is frequently associated with the development of ascending aortic aneurysm, even if the underlying mechanisms remain to be clarified. Here, we investigated if a deregulation of Notch1 signaling pathway and endothelial progenitor cells (EPCs) number is associated with BAV disease and an early ascending aortic aneurysm (AAA) onset. For this purpose, 70 subjects with BAV (M/F 50/20; mean age: 58.8 ± 14.8 years) and 70 subjects with tricuspid aortic valve (TAV) (M/F 35/35; mean age: 69.1 ± 12.8 years) and AAA complicated or not, were included. Interestingly, patients with AAA showed a significant increase in circulating Notch1 levels and EPC number than subjects without AAA. However, circulating Notch1 levels and EPC number were significantly lower in BAV subjects than TAV patients either in the presence or absence of AAA. Finally, Notch pathway was activated to a greater extent in aortic aneurysmatic portions with respect to healthy aortic fragments in both BAV and TAV patients. However, the expression of genes encoding components and ligands of Notch pathway in aortic tissues was significantly lower in BAV than TAV subjects. Our study demonstrates that BAV subjects are characterized by a significant decrease in both tissue and circulating levels of Notch pathway, and in blood EPC number than TAV patients, either in presence or absence of AAA disease.Carmela R. BalistreriFloriana CrapanzanoLeonardo SchironeAlberto AllegraCalogera PisanoGiovanni RuvoloMaurizio ForteErnesto GrecoElena CavarrettaAntonino G. M. MarulloSebastiano SciarrettaGiacomo FratiNature PortfolioarticleEndothelial Progenitor Cells (EPC)Ascending Aorta Aneurysm (AAA)Bicuspid Aortic Valve (BAV)Notch Signaling PathwayTricuspid Aortic Valve (TAV)MedicineRScienceQENScientific Reports, Vol 8, Iss 1, Pp 1-10 (2018)
institution DOAJ
collection DOAJ
language EN
topic Endothelial Progenitor Cells (EPC)
Ascending Aorta Aneurysm (AAA)
Bicuspid Aortic Valve (BAV)
Notch Signaling Pathway
Tricuspid Aortic Valve (TAV)
Medicine
R
Science
Q
spellingShingle Endothelial Progenitor Cells (EPC)
Ascending Aorta Aneurysm (AAA)
Bicuspid Aortic Valve (BAV)
Notch Signaling Pathway
Tricuspid Aortic Valve (TAV)
Medicine
R
Science
Q
Carmela R. Balistreri
Floriana Crapanzano
Leonardo Schirone
Alberto Allegra
Calogera Pisano
Giovanni Ruvolo
Maurizio Forte
Ernesto Greco
Elena Cavarretta
Antonino G. M. Marullo
Sebastiano Sciarretta
Giacomo Frati
Deregulation of Notch1 pathway and circulating endothelial progenitor cell (EPC) number in patients with bicuspid aortic valve with and without ascending aorta aneurysm
description Abstract Bicuspid aortic valve (BAV) is frequently associated with the development of ascending aortic aneurysm, even if the underlying mechanisms remain to be clarified. Here, we investigated if a deregulation of Notch1 signaling pathway and endothelial progenitor cells (EPCs) number is associated with BAV disease and an early ascending aortic aneurysm (AAA) onset. For this purpose, 70 subjects with BAV (M/F 50/20; mean age: 58.8 ± 14.8 years) and 70 subjects with tricuspid aortic valve (TAV) (M/F 35/35; mean age: 69.1 ± 12.8 years) and AAA complicated or not, were included. Interestingly, patients with AAA showed a significant increase in circulating Notch1 levels and EPC number than subjects without AAA. However, circulating Notch1 levels and EPC number were significantly lower in BAV subjects than TAV patients either in the presence or absence of AAA. Finally, Notch pathway was activated to a greater extent in aortic aneurysmatic portions with respect to healthy aortic fragments in both BAV and TAV patients. However, the expression of genes encoding components and ligands of Notch pathway in aortic tissues was significantly lower in BAV than TAV subjects. Our study demonstrates that BAV subjects are characterized by a significant decrease in both tissue and circulating levels of Notch pathway, and in blood EPC number than TAV patients, either in presence or absence of AAA disease.
format article
author Carmela R. Balistreri
Floriana Crapanzano
Leonardo Schirone
Alberto Allegra
Calogera Pisano
Giovanni Ruvolo
Maurizio Forte
Ernesto Greco
Elena Cavarretta
Antonino G. M. Marullo
Sebastiano Sciarretta
Giacomo Frati
author_facet Carmela R. Balistreri
Floriana Crapanzano
Leonardo Schirone
Alberto Allegra
Calogera Pisano
Giovanni Ruvolo
Maurizio Forte
Ernesto Greco
Elena Cavarretta
Antonino G. M. Marullo
Sebastiano Sciarretta
Giacomo Frati
author_sort Carmela R. Balistreri
title Deregulation of Notch1 pathway and circulating endothelial progenitor cell (EPC) number in patients with bicuspid aortic valve with and without ascending aorta aneurysm
title_short Deregulation of Notch1 pathway and circulating endothelial progenitor cell (EPC) number in patients with bicuspid aortic valve with and without ascending aorta aneurysm
title_full Deregulation of Notch1 pathway and circulating endothelial progenitor cell (EPC) number in patients with bicuspid aortic valve with and without ascending aorta aneurysm
title_fullStr Deregulation of Notch1 pathway and circulating endothelial progenitor cell (EPC) number in patients with bicuspid aortic valve with and without ascending aorta aneurysm
title_full_unstemmed Deregulation of Notch1 pathway and circulating endothelial progenitor cell (EPC) number in patients with bicuspid aortic valve with and without ascending aorta aneurysm
title_sort deregulation of notch1 pathway and circulating endothelial progenitor cell (epc) number in patients with bicuspid aortic valve with and without ascending aorta aneurysm
publisher Nature Portfolio
publishDate 2018
url https://doaj.org/article/cb1af62bbb1a40608db4adefa71c4b88
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