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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...
Autores principales: | Halabi, Carmen M., Broekelmann, Thomas J., Lin, Michelle, Lee, Vivian S., Chu, Mon-Li, Mecham, Robert P. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Association for the Advancement of Science
2017
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Materias: | |
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 |
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