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Nuclear entrapment and extracellular depletion of PCOLCE is associated with muscle degeneration in oculopharyngeal muscular dystrophy
BACKGROUND: Muscle fibrosis characterizes degenerated muscles in muscular dystrophies and in late onset myopathies. Fibrotic muscles often exhibit thickening of the extracellular matrix (ECM). The molecular regulation of this process is not fully understood. In oculopharyngeal muscular dystrophy (OP...
Autores principales: | Raz, Vered, Sterrenburg, Ellen, Routledge, Samantha, Venema, Andrea, van der Sluijs, Barbara M, Trollet, Capucine, Dickson, George, van Engelen, Baziel GM, van der Maarel, Silvère M, Antoniou, Michael N |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3717027/ https://www.ncbi.nlm.nih.gov/pubmed/23815790 http://dx.doi.org/10.1186/1471-2377-13-70 |
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