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Transcriptome Analysis of Ullrich Congenital Muscular Dystrophy Fibroblasts Reveals a Disease Extracellular Matrix Signature and Key Molecular Regulators

BACKGROUND: Collagen VI related myopathies encompass a range of phenotypes with involvement of skeletal muscle, skin and other connective tissues. They represent a severe and relatively common form of congenital disease for which there is no treatment. Collagen VI in skeletal muscle and skin is prod...

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Detalles Bibliográficos
Autores principales: Paco, Sonia, Casserras, Teresa, Rodríguez, Maria Angels, Jou, Cristina, Puigdelloses, Montserrat, Ortez, Carlos I., Diaz-Manera, Jordi, Gallardo, Eduardo, Colomer, Jaume, Nascimento, Andrés, Kalko, Susana G., Jimenez-Mallebrera, Cecilia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4686057/
https://www.ncbi.nlm.nih.gov/pubmed/26670220
http://dx.doi.org/10.1371/journal.pone.0145107