Deficient Circumferential Growth is the Primary Determinant of Aortic Obstruction Attributable to Partial Elastin Deficiency

OBJECTIVE: Williams syndrome (WS) is characterized by obstructive aortopathy attributable to heterozygous loss of ELN, the gene encoding elastin. Lesions are thought to result primarily from excessive smooth muscle cell (SMC) proliferation and consequent medial expansion, although an initially small...

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Veröffentlicht in:Arterioscler Thromb Vasc Biol
Hauptverfasser: Jiao, Yang, Li, Guangxin, Korneva, Arina, Caulk, Alexander W., Qin, Lingfeng, Bersi, Matthew R., Li, Qingle, Li, Wei, Mecham, Robert P., Humphrey, Jay D., Tellides, George
Format: Artigo
Sprache:Inglês
Veröffentlicht: 2017
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Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5501334/
https://ncbi.nlm.nih.gov/pubmed/28254817
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1161/ATVBAHA.117.309079
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