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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Huvudupphovsmän: 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
Materialtyp: Artigo
Språk:Inglês
Publicerad: BioMed Central 2013
Ämnen:
Research Article
Länkar: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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