Fibulin-4 is essential for maintaining arterial wall integrity in conduit but not muscular arteries

Homozygous or compound heterozygous mutations in fibulin-4 (FBLN4) lead to autosomal recessive cutis laxa type 1B (ARCL1B), a multisystem disorder characterized by significant cardiovascular abnormalities, including abnormal elastin assembly, arterial tortuosity, and aortic aneurysms. We sought to d...

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Detalles Bibliográficos
Autores principales: Halabi, Carmen M., Broekelmann, Thomas J., Lin, Michelle, Lee, Vivian S., Chu, Mon-Li, Mecham, Robert P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Association for the Advancement of Science 2017
Materias:
Research Articles
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5415335/
https://www.ncbi.nlm.nih.gov/pubmed/28508064
http://dx.doi.org/10.1126/sciadv.1602532

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5415335/
https://www.ncbi.nlm.nih.gov/pubmed/28508064
http://dx.doi.org/10.1126/sciadv.1602532

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