The small compound Icerguastat reduces muscle defects in oculopharyngeal muscular dystrophy through the PERK pathway of the unfolded protein response

Oculopharyngeal muscular dystrophy (OPMD) is an autosomal dominant disease characterized by the progressive degeneration of specific muscles. OPMD is due to a mutation in the gene encoding poly(A) binding protein nuclear 1 (PABPN1) leading to a stretch of 11 to 18 alanines at N-terminus of the prote...

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Detalles Bibliográficos
Autores principales: Naït-Saïdi, Rima, Chartier, Aymeric, Abgueguen, Emmanuelle, Guédat, Philippe, Simonelig, Martine
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Royal Society 2023
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10090878/
https://www.ncbi.nlm.nih.gov/pubmed/37042114
http://dx.doi.org/10.1098/rsob.230008

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10090878/
https://www.ncbi.nlm.nih.gov/pubmed/37042114
http://dx.doi.org/10.1098/rsob.230008

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