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Arginine-rich C9ORF72 ALS proteins stall ribosomes in a manner distinct from a canonical ribosome-associated quality control substrate

Hexanucleotide expansion mutations in C9ORF72 are a frequent cause of amyotrophic lateral sclerosis. We previously reported that long arginine-rich dipeptide repeats (DPRs), mimicking abnormal proteins expressed from the hexanucleotide expansion, caused translation stalling when expressed in cell cu...

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Detalles Bibliográficos
Autores principales: Kriachkov, Viacheslav, Ormsby, Angelique R., Kusnadi, Eric P., McWilliam, Hamish E.G., Mintern, Justine D., Amarasinghe, Shanika L., Ritchie, Matthew E., Furic, Luc, Hatters, Danny M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9830226/
https://www.ncbi.nlm.nih.gov/pubmed/36481270
http://dx.doi.org/10.1016/j.jbc.2022.102774