Poly-glycine–alanine exacerbates C9orf72 repeat expansion-mediated DNA damage via sequestration of phosphorylated ATM and loss of nuclear hnRNPA3

Repeat expansion in C9orf72 causes amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Expanded sense and antisense repeat RNA transcripts in C9orf72 are translated into five dipeptide-repeat proteins (DPRs) in an AUG-independent manner. We previously identified the heterogeneous ri...

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Detalles Bibliográficos
Autores principales: Nihei, Yoshihiro, Mori, Kohji, Werner, Georg, Arzberger, Thomas, Zhou, Qihui, Khosravi, Barham, Japtok, Julia, Hermann, Andreas, Sommacal, Andreas, Weber, Markus, Kamp, Frits, Nuscher, Brigitte, Edbauer, Dieter, Haass, Christian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2019
Materias:
Original Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6942035/
https://www.ncbi.nlm.nih.gov/pubmed/31642962
http://dx.doi.org/10.1007/s00401-019-02082-0

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