Modeling Oculopharyngeal Muscular Dystrophy in Myotube Cultures Reveals Reduced Accumulation of Soluble Mutant PABPN1 Protein

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disease caused by an alanine tract expansion mutation in poly(A) binding protein nuclear 1 (expPABPN1). To model OPMD in a myogenic and physiological context, we generated mouse myoblast cell clones stably expressing either human wil...

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Detalhes bibliográficos
Main Authors: Raz, Vered, Routledge, Samantha, Venema, Andrea, Buijze, Hellen, van der Wal, Erik, Anvar, SeyedYahya, Straasheijm, Kirsten R., Klooster, Rinse, Antoniou, Michael, van der Maarel, Silvère M.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Investigative Pathology 2011
Assuntos:
Regular Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3181361/
https://ncbi.nlm.nih.gov/pubmed/21854744
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ajpath.2011.06.044
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