Multiple pathways of toxicity induced by C9orf72 dipeptide repeat aggregates and G4C2 RNA in a cellular model

The most frequent genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia is a G4C2 repeat expansion in the C9orf72 gene. This expansion gives rise to translation of aggregating dipeptide repeat (DPR) proteins, including poly-GA as the most abundant species. However, gain of toxic...

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Bibliographic Details
Published in:eLife
Main Authors: Frédéric Frottin, Manuela Pérez-Berlanga, F Ulrich Hartl, Mark S Hipp
Format: Article
Language:English
Published: eLife Sciences Publications Ltd 2021-06-01
Subjects:
quality control
protein aggregation
neurodegeneration
Online Access:https://elifesciences.org/articles/62718

Internet

https://elifesciences.org/articles/62718

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