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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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| Pubblicato in: | Arterioscler Thromb Vasc Biol |
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| Autori principali: | , , , , , , , , , , |
| Natura: | Artigo |
| Lingua: | Inglês |
| Pubblicazione: |
2017
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| Soggetti: | |
| Accesso online: | 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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