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http://hdl.handle.net/2445/174888
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DC Field | Value | Language |
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dc.contributor.author | Perrucci, Gianluca L. | - |
dc.contributor.author | Rurali, Erica | - |
dc.contributor.author | Corlianò, Maria | - |
dc.contributor.author | Balzo, Maria | - |
dc.contributor.author | Piccoli, Michela | - |
dc.contributor.author | Moschetta, Donato | - |
dc.contributor.author | Pini, Alessandro | - |
dc.contributor.author | Gaetano, Raffaella | - |
dc.contributor.author | Antona, Carlo | - |
dc.contributor.author | Egea Guri, Gustavo | - |
dc.contributor.author | Fischer, Gunter | - |
dc.contributor.author | Maleševic ́, Miroslav | - |
dc.contributor.author | Alamanni, Francesco | - |
dc.contributor.author | Cogliati, Elisa | - |
dc.contributor.author | Paolin, Adolfo | - |
dc.contributor.author | Pompilio, Giulio | - |
dc.contributor.author | Nigro, Patrizia | - |
dc.date.accessioned | 2021-03-10T14:19:10Z | - |
dc.date.available | 2021-03-10T14:19:10Z | - |
dc.date.issued | 2020-01-08 | - |
dc.identifier.issn | 2073-4409 | - |
dc.identifier.uri | http://hdl.handle.net/2445/174888 | - |
dc.description.abstract | Background: Marfan syndrome (MFS) is a genetic disease, characterized by thoracic aortic aneurysm (TAA), which treatment is to date purely surgical. Understanding of novel molecular targets is mandatory to unveil e ective pharmacological approaches. Cyclophilin A (CyPA) and its receptor EMMPRIN are associated with several cardiovascular diseases, including abdominal aortic aneurysm. Here, we envisioned the contribution of CyPA/EMMPRIN axis in MFS-related TAA. Methods: We obtained thoracic aortic samples from healthy controls (HC) and MFS patients' aortas and then isolated vascular smooth muscle cells (VSMC) from the aortic wall. Results: our findings revealed that MFS aortic tissue samples isolated from the dilated zone of aorta showed higher expression levels of EMMPRIN vs. MFS non-dilated aorta and HC. Interestingly, angiotensin II significantly stimulated CyPA secretion in MFS-derived VSMC (MFS-VSMC). CyPA treatment on MFS-VSMC led to increased levels of EMMPRIN and other MFS-associated pro-fibrotic mediators, such as TGF- 1 and collagen I. These molecules were downregulated by in vitro treatment with CyPA inhibitor MM284. Our results suggest that CyPA/EMMPRIN axis is involved in MFS-related TAA development, since EMMPRIN is upregulated in the dilated zone of MFS patients' TAA and the inhibition of its ligand, CyPA, downregulated EMMPRIN and MFS-related markers in MFS-VSMC. Conclusions: these insights suggest both a novel detrimental role for CyPA/EMMPRIN axis and its inhibition as a potential therapeutic strategy for MFS-related TAA treatment. | - |
dc.format.extent | 18 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | MDPI | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.3390/cells9010154 | - |
dc.relation.ispartof | Cells, 2020, vol. 9, num. 154 | - |
dc.relation.uri | https://doi.org/10.3390/cells9010154 | - |
dc.rights | cc-by (c) Perrucci, Gianluca L. et al., 2020 | - |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/es | - |
dc.source | Articles publicats en revistes (Biomedicina) | - |
dc.subject.classification | Aneurismes aòrtics | - |
dc.subject.classification | Genètica | - |
dc.subject.other | Aortic aneurysms | - |
dc.subject.other | Genetics | - |
dc.title | Cyclophilin A/EMMPRIN Axis Is Involved in Pro-Fibrotic Processes Associated with Thoracic Aortic Aneurysm of Marfan Syndrome Patients | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 699229 | - |
dc.date.updated | 2021-03-10T14:19:10Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 31936351 | - |
Appears in Collections: | Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer) Articles publicats en revistes (Biomedicina) |
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699229.pdf | 4.28 MB | Adobe PDF | View/Open |
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