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Article

An amyloid-like cascade hypothesis for C9orf72 ALS/FTD

8 Nov 2015

Abstract excerpt

Expansion of a GGGGCC repeat in C9orf72 causes amyotrophic lateral sclerosis, frontotemporal dementia, or a combination of both. Bidirectional repeat transcripts sequester RNA-binding proteins into nuclear RNA foci. The repeat is translated into dipeptide repeat (DPR) proteins that are crucial for repeat-induced toxicity. DPRs inhibit the proteasome and sequester other proteins. These changes are accompanied by...

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PMID: 26555807
DOI: 10.1016/j.conb.2015.10.009

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