Abstract:The expansion of GGGGCC (G4C2) repeats in the noncoding region of C9orf72 is the most common genetic cause of familial and sporadic amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The repeat region is translated into five different dipeptide repeats (DPRs), of which the arginine-rich DPRs (R-DPRs) poly-GR (GRn) and poly-PR (PRn) are highly neurotoxic and are probably primarily responsible for the disease. Here, we characterized the protective effect against R-DPR toxicity of polystyrene … Show more
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