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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Detalhes bibliográficos
Publicado no:Arterioscler Thromb Vasc Biol
Main Authors: 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
Formato: Artigo
Idioma:Inglês
Publicado em: 2017
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Article
Acesso em linha: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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