14 research outputs found
Special Features of Computational Assessment of the Change in Shape of WWER-1000 Reactor Core Baffle in View of Irradiation-Induced Swelling
On the Correctness of the Well-Known Mathematical Model of Irradiation-Induced Swelling with the Influence of Stresses in the Problems of Elastic-Plastic Deformation Mechanics
Simple and Portable Magnetic Immunoassay for Rapid Detection and Sensitive Quantification of Plant Viruses
Body size and its implications upon resource utilization during human space exploration missions
Evidence on antimicrobial properties and mode of action of a chitosan obtained from crustacean exoskeletons on Pseudomonas syringae pv. tomato DC3000
Stenosis coexists with compromised α1-adrenergic contractions in the ascending aorta of a mouse model of Williams-Beuren syndrome
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.This work was supported by Ministerio de Ciencia e Innovación [[SAF2014-56111-R to FJA] and [SAF2016-78508-R (AEI/MINEICO/FEDER, UE) to VC]]; and Generalitat de Catalunya [2017-SGR-645 to FJA]