Expression of collagen VI α5 and α6 chains in human muscle and in Duchenne muscular dystrophy-related muscle fibrosis

Collagen VI is a major extracellular matrix (ECM) protein with a critical role in maintaining skeletal muscle functional integrity. Mutations in COL6A1, COL6A2 and COL6A3 genes cause Ullrich Congenital Muscular Dystrophy (UCMD), Bethlem Myopathy, and Myosclerosis. Moreover, Col6a1(−/−) mice and coll...

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Main Authors: Sabatelli, Patrizia, Gualandi, Francesca, Gara, Sudheer Kumar, Grumati, Paolo, Zamparelli, Alessandra, Martoni, Elena, Pellegrini, Camilla, Merlini, Luciano, Ferlini, Alessandra, Bonaldo, Paolo, Maraldi, Nadir Mario, Paulsson, Mats, Squarzoni, Stefano, Wagener, Raimund
Format: Artigo
Sprog:Inglês
Udgivet: Elsevier 2012
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3315014/
https://ncbi.nlm.nih.gov/pubmed/22226732
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.matbio.2011.12.003
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