Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits

Intronic GGGGCC repeat expansions in C9orf72 are the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Two major pathologies stemming from the hexanucleotide RNA expansions (HREs) have been identified in postmortem tissue: intracellular RNA foci and...

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Detalles Bibliográficos
Autores principales: Herranz-Martin, Saul, Chandran, Jayanth, Lewis, Katherine, Mulcahy, Padraig, Higginbottom, Adrian, Walker, Callum, Valenzuela, Isabel Martinez-Pena y, Jones, Ross A., Coldicott, Ian, Iannitti, Tommaso, Akaaboune, Mohammed, El-Khamisy, Sherif F., Gillingwater, Thomas H., Shaw, Pamela J., Azzouz, Mimoun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Company of Biologists Ltd 2017
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5536911/
https://www.ncbi.nlm.nih.gov/pubmed/28550099
http://dx.doi.org/10.1242/dmm.029892

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5536911/
https://www.ncbi.nlm.nih.gov/pubmed/28550099
http://dx.doi.org/10.1242/dmm.029892

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