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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Bibliographic Details
Main Authors: Gerhard Sengle, Valerie Carlberg, Sara F Tufa, Noe L Charbonneau, Silvia Smaldone, Eric J Carlson, Francesco Ramirez, Douglas R Keene, Lynn Y Sakai
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2015-06-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC4482570?pdf=render