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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Autori principali: 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
Natura: Artigo
Lingua:Inglês
Pubblicazione: BioMed Central 2013
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Research Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3717027/
https://ncbi.nlm.nih.gov/pubmed/23815790
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1471-2377-13-70
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