التفاصيل البيبلوغرافية
| العنوان: |
Stenosis coexists with compromised α1-adrenergic contractions in the ascending aorta of a mouse model of Williams-Beuren syndrome |
| المؤلفون: |
Jiménez Altayó, Francesc, Ortiz-Romero, Paula, Puertas Umbert, Lídia, Dantas, A. P, Pérez, Belén, Vila Calsina, Elisabet, D'Ocon, Pilar, Campuzano, Victoria |
| سنة النشر: |
2020 |
| المجموعة: |
Universitat Autònoma de Barcelona: Dipòsit Digital de Documents de la UAB |
| مصطلحات موضوعية: |
Valvular disease, Aortic diseases |
| الوصف: |
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. |
| نوع الوثيقة: |
article in journal/newspaper |
| وصف الملف: |
application/pdf |
| اللغة: |
English |
| تدمد: |
85078204 |
| العلاقة: |
Ministerio de Economía y Competitividad SAF2014-56111-R; Scientific reports; Vol. 10 (january 2020); https://ddd.uab.cat/record/252410Test; urn:10.1038/s41598-020-57803-3; urn:oai:ddd.uab.cat:252410; urn:pmcid:PMC6972706; urn:pmc-uid:6972706; urn:pmid:31965005; urn:oai:pubmedcentral.nih.gov:6972706; urn:oai:egreta.uab.cat:publications/ad4fd94c-e527-4c41-8efe-54ab111521a2; urn:scopus_id:85078204154 |
| الإتاحة: |
https://ddd.uab.cat/record/252410Test |
| حقوق: |
open access ; Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original. ; https://creativecommons.org/licenses/by/4.0Test/ |
| رقم الانضمام: |
edsbas.BC4F079F |
| قاعدة البيانات: |
BASE |
