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C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

An expanded GGGGCC repeat in C9orf72 is the most common genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis. A fundamental question is whether toxicity is driven by the repeat RNA itself and/or by dipeptide repeat proteins generated by repeat-associated, non-ATG translation. T...

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Detalhes bibliográficos
Publicado no:Science
Main Authors: Mizielinska, Sarah, Grönke, Sebastian, Niccoli, Teresa, Ridler, Charlotte E., Clayton, Emma L., Devoy, Anny, Moens, Thomas, Norona, Frances E., Woollacott, Ione O.C., Pietrzyk, Julian, Cleverley, Karen, Nicoll, Andrew J., Pickering-Brown, Stuart, Dols, Jacqueline, Cabecinha, Melissa, Hendrich, Oliver, Fratta, Pietro, Fisher, Elizabeth M.C., Partridge, Linda, Isaacs, Adrian M.
Formato: Artigo
Idioma:Inglês
Publicado em: 2014
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4944841/
https://ncbi.nlm.nih.gov/pubmed/25103406
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1126/science.1256800
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