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Tendon Extracellular Matrix Remodeling and Defective Cell Polarization in the Presence of Collagen VI Mutations

Mutations in collagen VI genes cause two major clinical myopathies, Bethlem myopathy (BM) and Ullrich congenital muscular dystrophy (UCMD), and the rarer myosclerosis myopathy. In addition to congenital muscle weakness, patients affected by collagen VI-related myopathies show axial and proximal join...

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Detalles Bibliográficos
Autores principales: Antoniel, Manuela, Traina, Francesco, Merlini, Luciano, Andrenacci, Davide, Tigani, Domenico, Santi, Spartaco, Cenni, Vittoria, Sabatelli, Patrizia, Faldini, Cesare, Squarzoni, Stefano
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7072441/
https://www.ncbi.nlm.nih.gov/pubmed/32053901
http://dx.doi.org/10.3390/cells9020409