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C9orf72 arginine-rich dipeptide proteins interact with ribosomal proteins in vivo to induce a toxic translational arrest that is rescued by eIF1A
A GGGGCC hexanucleotide repeat expansion within the C9orf72 gene is the most common genetic cause of both amyotrophic lateral sclerosis and frontotemporal dementia. Sense and antisense repeat-containing transcripts undergo repeat-associated non-AUG-initiated translation to produce five dipeptide pro...
Autores principales: | Moens, Thomas G., Niccoli, Teresa, Wilson, Katherine M., Atilano, Magda L., Birsa, Nicol, Gittings, Lauren M., Holbling, Benedikt V., Dyson, Miranda C., Thoeng, Annora, Neeves, Jacob, Glaria, Idoia, Yu, Lu, Bussmann, Julia, Storkebaum, Erik, Pardo, Mercedes, Choudhary, Jyoti S., Fratta, Pietro, Partridge, Linda, Isaacs, Adrian M. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6514073/ https://www.ncbi.nlm.nih.gov/pubmed/30604225 http://dx.doi.org/10.1007/s00401-018-1946-4 |
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