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Marfan syndrome; a connective tissue disease at the crossroads of mechanotransduction, TGFβ signaling and cell stemness

Mutations in fibrillin-1 cause Marfan syndrome (MFS), the most common heritable disorder of connective tissue. Fibrillin-1 assemblies (microfibrils and elastic fibers) represent a unique dual-function component of the architectural matrix. The first role is structural for they endow tissues with ten...

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Detalhes bibliográficos
Publicado no:Matrix Biol
Main Authors: Ramirez, Francesco, Caescu, Cristina, Wondimu, Elisabeth, Galatioto, Josephine
Formato: Artigo
Idioma:Inglês
Publicado em: 2017
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5797509/
https://ncbi.nlm.nih.gov/pubmed/28782645
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.matbio.2017.07.004
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