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New Insights into the Assembly of Extracellular Microfibrils from the Analysis of the Fibrillin 1 Mutation in the Tight skin Mouse

The Tight skin (Tsk) mutation is a duplication of the mouse fibrillin 1 (Fbn1) gene that results in a larger (418 kD) than normal (350 kD) protein; Tsk/+ mice display increased connective tissue, bone overgrowth, and lung emphysema. Lung emphysema, bone overgrowth, and vascular complications are the...

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Autores principales: Gayraud, Barbara, Keene, Douglas R., Sakai, Lynn Y., Ramirez, Francesco
Formato: Artigo
Lenguaje:Inglês
Publicado: The Rockefeller University Press 2000
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC2175205/
https://ncbi.nlm.nih.gov/pubmed/10931876
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