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

BACKGROUND: Williams 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. METHODS:...

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Detalles Bibliográficos
Autores principales: Knutsen, Russell H., Gober, Leah M., Kronquist, Elise K., Kaur, Maninder, Donahue, Danielle R., Springer, Danielle, Yu, Zu Xi, Chen, Marcus Y., Fu, Yi-Ping, Choobdar, Feri, Nguyen, My-Le, Osgood, Sharon, Freeman, Joy L., Raja, Neelam, Levin, Mark D., Kozel, Beth A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Cardiovascular Medicine
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9160528/
https://www.ncbi.nlm.nih.gov/pubmed/35665242
http://dx.doi.org/10.3389/fcvm.2022.886813

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