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http://hdl.handle.net/2445/162717
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DC Field | Value | Language |
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dc.contributor.author | Jiménez Altayó, Francesc | - |
dc.contributor.author | Ortiz Romero, Paula | - |
dc.contributor.author | Puertas Umbert, Lídia | - |
dc.contributor.author | Dantas, Ana Paula | - |
dc.contributor.author | Pérez, Belén | - |
dc.contributor.author | Vila, Elisabet | - |
dc.contributor.author | D'Ocon, Pilar | - |
dc.contributor.author | Campuzano Uceda, María Victoria | - |
dc.date.accessioned | 2020-05-27T18:21:07Z | - |
dc.date.available | 2020-05-27T18:21:07Z | - |
dc.date.issued | 2020-01-21 | - |
dc.identifier.issn | 2045-2322 | - |
dc.identifier.uri | http://hdl.handle.net/2445/162717 | - |
dc.description.abstract | Williams-Beuren syndrome (WBS) is a rare disorder caused by a heterozygous deletion of 26-28 contiguous genes that affects the brain and cardiovascular system. Here, we investigated whether WBS affects aortic structure and function in the complete deletion (CD) mouse model harbouring the most common deletion found in WBS patients. Thoracic aortas from 3-4 months-old male CD mice and wild-type littermates were mounted in wire myographs or were processed for histomorphometrical analysis. Nitric oxide synthase (NOS) isoforms and oxidative stress levels were assessed. Ascending aortas from young adult CD mice showed moderate (50%) luminal stenosis, whereas endothelial function and oxidative stress were comparable to wild-type. CD mice showed greater contractions to KCl. However, α1-adrenergic contractions to phenylephrine, but not with a thromboxane analogue, were compromised. Decreased phenylephrine responses were not affected by selective inducible NOS blockade with 1400 W, but were prevented by the non-selective NOS inhibitor L-NAME and the selective neuronal NOS inhibitor SMTC. Consistently, CD mice showed increased neuronal NOS expression in aortas. Overall, aortic stenosis in CD mice coexists with excessive nNOS-derived NO signaling that compromises ascending aorta α1-adrenergic contractions. We suggest that increased neuronal NOS signaling may act as a physiological 'brake' against the detrimental effects of stenosis. | - |
dc.format.extent | 12 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | Nature Publishing Group | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1038/s41598-020-57803-3 | - |
dc.relation.ispartof | Scientific Reports, 2020, vol. 10, num. 889 | - |
dc.relation.uri | https://doi.org/10.1038/s41598-020-57803-3 | - |
dc.rights | cc-by (c) Jiménez Altayó, Francesc et al., 2020 | - |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/es | - |
dc.source | Articles publicats en revistes (Biomedicina) | - |
dc.subject.classification | Models animals en la investigació | - |
dc.subject.classification | Malalties hereditàries | - |
dc.subject.classification | Malalties cardiovasculars | - |
dc.subject.classification | Estenosi | - |
dc.subject.other | Animal models in research | - |
dc.subject.other | Genetic diseases | - |
dc.subject.other | Cardiovascular diseases | - |
dc.subject.other | Stenosis | - |
dc.title | Stenosis coexists with compromised α1-adrenergic contractions in the ascending aorta of a mouse model of Williams-Beuren syndrome | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 701211 | - |
dc.date.updated | 2020-05-27T18:21:07Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 31965005 | - |
Appears in Collections: | Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer) Articles publicats en revistes (Biomedicina) |
Files in This Item:
File | Description | Size | Format | |
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701211.pdf | 1.73 MB | Adobe PDF | View/Open |
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