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Knocking out C9ORF72 Exacerbates Axonal Trafficking Defects Associated with Hexanucleotide Repeat Expansion and Reduces Levels of Heat Shock Proteins
In amyotrophic lateral sclerosis (ALS) motor neurons (MNs) undergo dying-back, where the distal axon degenerates before the soma. The hexanucleotide repeat expansion (HRE) in C9ORF72 is the most common genetic cause of ALS, but the mechanism of pathogenesis is largely unknown with both gain- and los...
Autores principales: | Abo-Rady, Masin, Kalmbach, Norman, Pal, Arun, Schludi, Carina, Janosch, Antje, Richter, Tanja, Freitag, Petra, Bickle, Marc, Kahlert, Anne-Karin, Petri, Susanne, Stefanov, Stefan, Glass, Hannes, Staege, Selma, Just, Walter, Bhatnagar, Rajat, Edbauer, Dieter, Hermann, Andreas, Wegner, Florian, Sterneckert, Jared L. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7066330/ https://www.ncbi.nlm.nih.gov/pubmed/32084385 http://dx.doi.org/10.1016/j.stemcr.2020.01.010 |
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