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BV-2 Microglial Cells Overexpressing C9orf72 Hexanucleotide Repeat Expansion Produce DPR Proteins and Show Normal Functionality but No RNA Foci

Hexanucleotide repeat expansion (HRE) in the chromosome 9 open-reading frame 72 (C9orf72) gene is the most common genetic cause underpinning frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). It leads to the accumulation of toxic RNA foci and various dipeptide repeat (...

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Detalles Bibliográficos
Autores principales: Rostalski, Hannah, Hietanen, Tomi, Leskelä, Stina, Behánová, Andrea, Abdollahzadeh, Ali, Wittrahm, Rebekka, Mäkinen, Petra, Huber, Nadine, Hoffmann, Dorit, Solje, Eino, Remes, Anne M., Natunen, Teemu, Takalo, Mari, Tohka, Jussi, Hiltunen, Mikko, Haapasalo, Annakaisa
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7573144/
https://www.ncbi.nlm.nih.gov/pubmed/33123074
http://dx.doi.org/10.3389/fneur.2020.550140