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Mitochondrial Dysfunction Reveals the Role of mRNA Poly(A) Tail Regulation in Oculopharyngeal Muscular Dystrophy Pathogenesis

Oculopharyngeal muscular dystrophy (OPMD), a late-onset disorder characterized by progressive degeneration of specific muscles, results from the extension of a polyalanine tract in poly(A) binding protein nuclear 1 (PABPN1). While the roles of PABPN1 in nuclear polyadenylation and regulation of alte...

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Detalles Bibliográficos
Autores principales: Chartier, Aymeric, Klein, Pierre, Pierson, Stéphanie, Barbezier, Nicolas, Gidaro, Teresa, Casas, François, Carberry, Steven, Dowling, Paul, Maynadier, Laurie, Bellec, Maëlle, Oloko, Martine, Jardel, Claude, Moritz, Bodo, Dickson, George, Mouly, Vincent, Ohlendieck, Kay, Butler-Browne, Gillian, Trollet, Capucine, Simonelig, Martine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4376527/
https://www.ncbi.nlm.nih.gov/pubmed/25816335
http://dx.doi.org/10.1371/journal.pgen.1005092