PABPN1 aggregation is driven by Ala expansion and poly(A)-RNA binding, leading to CFIm25 sequestration that impairs alternative polyadenylation

Poly(A)-binding protein nuclear 1 (PABPN1) is an RNA-binding protein localized in nuclear speckles, while its alanine (Ala)-expanded variants accumulate as intranuclear aggregates in oculopharyngeal muscular dystrophy. The factors that drive PABPN1 aggregation and its cellular consequences remain la...

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Detalles Bibliográficos
Autores principales: Guan, Wen-Liang, Jiang, Lei-Lei, Yin, Xiao-Fang, Hu, Hong-Yu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2023
Materias:
Research Article Collection: Molecular Bases of Disease
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10403730/
https://www.ncbi.nlm.nih.gov/pubmed/37422193
http://dx.doi.org/10.1016/j.jbc.2023.105019

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10403730/
https://www.ncbi.nlm.nih.gov/pubmed/37422193
http://dx.doi.org/10.1016/j.jbc.2023.105019

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