The Fbn1 gene variant governs passive ascending aortic mechanics in the mgΔlpn mouse model of Marfan syndrome when superimposed to perlecan haploinsufficiency

The Fbn1 gene variant governs passive ascending aortic mechanics in the mgΔlpn mouse model of Marfan syndrome when superimposed to perlecan haploinsufficiency

IntroductionAscending thoracic aortic aneurysms arise from pathological tissue remodeling that leads to abnormal wall dilation and increases the risk of fatal dissection/rupture. Large variability in disease manifestations across family members who carry a causative genetic variant for thoracic aort...

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Bibliographic Details
Main Authors: Samar A. Tarraf, Rodrigo Barbosa de Souza, Ashley Herrick, Lygia V. Pereira, Chiara Bellini
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-03-01
Series:Frontiers in Cardiovascular Medicine
Subjects:
ascending aneurysm
biomechanics
Marfan syndrome
fibrillin-1
perlecan
Online Access:https://www.frontiersin.org/articles/10.3389/fcvm.2024.1319164/full

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