Elastin Insufficiency Confers Proximal and Distal Pulmonary Vasculopathy in Mice, Partially Remedied by the KATP Channel Opener Minoxidil: Considerations and Cautions for the Treatment of People With Williams-Beuren Syndrome

Elastin Insufficiency Confers Proximal and Distal Pulmonary Vasculopathy in Mice, Partially Remedied by the KATP Channel Opener Minoxidil: Considerations and Cautions for the Treatment of People With Williams-Beuren Syndrome

BackgroundWilliams Beuren syndrome (WBS) is a recurrent microdeletion disorder that removes one copy of elastin (ELN), resulting in large artery vasculopathy. Early stenosis of the pulmonary vascular tree is common, but few data are available on longer-term implications of the condition.MethodsCompu...

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Bibliographic Details
Main Authors: Russell H. Knutsen, Leah M. Gober, Elise K. Kronquist, Maninder Kaur, Danielle R. Donahue, Danielle Springer, Zu Xi Yu, Marcus Y. Chen, Yi-Ping Fu, Feri Choobdar, My-Le Nguyen, Sharon Osgood, Joy L. Freeman, Neelam Raja, Mark D. Levin, Beth A. Kozel
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-05-01
Series:Frontiers in Cardiovascular Medicine
Subjects:
pulmonary
elastin
minoxidil
Williams syndrome
vasculopathy
pulmonary artery
Online Access:https://www.frontiersin.org/articles/10.3389/fcvm.2022.886813/full

Internet

https://www.frontiersin.org/articles/10.3389/fcvm.2022.886813/full

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