Matrisome and Immune Pathways Contribute to Extreme Vascular Outcomes in Williams–Beuren Syndrome

Matrisome and Immune Pathways Contribute to Extreme Vascular Outcomes in Williams–Beuren Syndrome

Background Supravalvar aortic stenosis (SVAS) is a characteristic feature of Williams–Beuren syndrome (WBS). Its severity varies: ~20% of people with Williams–Beuren syndrome have SVAS requiring surgical intervention, whereas ~35% have no appreciable SVAS. The remaining individuals have SVAS of inte...

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Bibliographic Details
Main Authors: Delong Liu, Charles J. Billington, Neelam Raja, Zoe C. Wong, Mark D. Levin, Wulfgang Resch, Camille Alba, Daniel N. Hupalo, Elisa Biamino, Maria Francesca Bedeschi, Maria Cristina Digilio, Gabriella Maria Squeo, Roberta Villa, Pheobe C. R. Parrish, Russell H. Knutsen, Sharon Osgood, Joy A. Freeman, Clifton L. Dalgard, Giuseppe Merla, Barbara R. Pober, Carolyn B. Mervis, Amy E. Roberts, Colleen A. Morris, Lucy R. Osborne, Beth A. Kozel
Format: Article
Language:English
Published: Wiley 2024-02-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
adaptive/innate immune system
elastin (ELN)
extreme phenotype
pathway analysis
supravalvar aortic stenosis
Williams–Beuren syndrome
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.123.031377

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https://www.ahajournals.org/doi/10.1161/JAHA.123.031377

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