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Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice

Fibrillins are large extracellular macromolecules that polymerize to form the backbone structure of connective tissue microfibrils. Mutations in the gene for fibrillin-1 cause the Marfan syndrome, while mutations in the gene for fibrillin-2 cause Congenital Contractural Arachnodactyly. Both are auto...

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Detalles Bibliográficos
Autores principales: Sengle, Gerhard, Carlberg, Valerie, Tufa, Sara F., Charbonneau, Noe L., Smaldone, Silvia, Carlson, Eric J., Ramirez, Francesco, Keene, Douglas R., Sakai, Lynn Y.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4482570/
https://www.ncbi.nlm.nih.gov/pubmed/26114882
http://dx.doi.org/10.1371/journal.pgen.1005340