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The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains

An expanded GGGGCC hexanucleotide in C9ORF72 (C9) is the most frequent known cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). It has been proposed that expanded transcripts adopt G-quadruplex (G-Q) structures and associate with proteins, but whether this occurs and con...

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Detalles Bibliográficos
Autores principales: Conlon, Erin G, Lu, Lei, Sharma, Aarti, Yamazaki, Takashi, Tang, Timothy, Shneider, Neil A, Manley, James L
Formato: Online Artículo Texto
Lenguaje:English
Publicado: eLife Sciences Publications, Ltd 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5050020/
https://www.ncbi.nlm.nih.gov/pubmed/27623008
http://dx.doi.org/10.7554/eLife.17820