Activation of the ubiquitin-proteasome system contributes to oculopharyngeal muscular dystrophy through muscle atrophy

Oculopharyngeal muscular dystrophy (OPMD) is a late-onset disorder characterized by progressive weakness and degeneration of specific muscles. OPMD is due to extension of a polyalanine tract in poly(A) binding protein nuclear 1 (PABPN1). Aggregation of the mutant protein in muscle nuclei is a hallma...

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Detalles Bibliográficos
Autores principales: Ribot, Cécile, Soler, Cédric, Chartier, Aymeric, Al Hayek, Sandy, Naït-Saïdi, Rima, Barbezier, Nicolas, Coux, Olivier, Simonelig, Martine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2022
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8791501/
https://www.ncbi.nlm.nih.gov/pubmed/35025870
http://dx.doi.org/10.1371/journal.pgen.1010015

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8791501/
https://www.ncbi.nlm.nih.gov/pubmed/35025870
http://dx.doi.org/10.1371/journal.pgen.1010015

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