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...

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Detalhes bibliográficos
Publicado no:Stem Cell Reports
Main Authors: 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.
Formato: Artigo
Idioma:Inglês
Publicado em: Elsevier 2020
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7066330/
https://ncbi.nlm.nih.gov/pubmed/32084385
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.stemcr.2020.01.010
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