Antisense Proline-Arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death

Expanded GGGGCC nucleotide repeats within the C9ORF72 gene are the most common genetic mutation associated with both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Sense and antisense transcripts of these expansions are translated to form five dipeptide repeat proteins (DRPs)...

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Publicat a:Neuron
Autors principals: Wen, Xinmei, Tan, Wenzhi, Westergard, Thomas, Krishnamurthy, Karthik, ShamamandriMarkandaiah, Shashirekha, Shi, Yingxiao, Lin, Shaoyu, Shneider, Neil A., Monaghan, John, Pandey, Udai B., Pasinelli, Piera, Ichida, Justin K., Trotti, Davide
Format: Artigo
Idioma:Inglês
Publicat: 2014
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4632245/
https://ncbi.nlm.nih.gov/pubmed/25521377
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.neuron.2014.12.010
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