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Inducible expression of human C9ORF72 36× G(4)C(2) hexanucleotide repeats is sufficient to cause RAN translation and rapid muscular atrophy in mice

The hexanucleotide G(4)C(2) repeat expansion in the first intron of the C9ORF72 gene accounts for the majority of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) cases. Numerous studies have indicated the toxicity of dipeptide repeats (DPRs), which are produced via repeat-assoc...

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Detalles Bibliográficos
Autores principales:	Riemslagh, Fréderike W., van der Toorn, Esmay C., Verhagen, Rob F. M., Maas, Alex, Bosman, Laurens W. J., Hukema, Renate K., Willemsen, Rob
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	The Company of Biologists Ltd 2021
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7903916/ https://www.ncbi.nlm.nih.gov/pubmed/33431483 http://dx.doi.org/10.1242/dmm.044842

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