Lineage-specific events underlie aortic root aneurysm pathogenesis in Loeys-Dietz syndrome

The aortic root is the predominant site for development of aneurysm caused by heterozygous loss-of-function mutations in positive effectors of the transforming growth factor-β (TGF-β) pathway. Using a mouse model of Loeys-Dietz syndrome (LDS) that carries a heterozygous kinase-inactivating mutation...

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Publicado en:J Clin Invest
Autores principales: MacFarlane, Elena Gallo, Parker, Sarah J., Shin, Joseph Y., Kang, Benjamin E., Ziegler, Shira G., Creamer, Tyler J., Bagirzadeh, Rustam, Bedja, Djahida, Chen, Yichun, Calderon, Juan F., Weissler, Katherine, Frischmeyer-Guerrerio, Pamela A., Lindsay, Mark E., Habashi, Jennifer P., Dietz, Harry C.
Formato: Artigo
Lenguaje:Inglês
Publicado: American Society for Clinical Investigation 2019
Materias:
Research Article
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC6355234/
https://ncbi.nlm.nih.gov/pubmed/30614814
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI123547
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