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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...

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2013
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