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Inducible expression of human <i>C9ORF72</i> 36x G <sub>4</sub> C <sub>2</sub> hexanucleotide repeats is sufficient to cause RAN translation and rapid muscular atrophy in mice

2020-09-16

Abstract excerpt

The hexanucleotide G 4 C 2 repeat expansion in the first intron of the C9ORF72 gene explains the majority of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) cases. Numerous studies have indicated the toxicity of dipeptide repeats (DPRs) which are produced via repeat-associated non-AUG (RAN) translation from the repeat expansion and accumulate in the brain of C9FTD/ALS patients. Mouse model...

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Literature Corpus work
9cb07746-8dd4-511f-9678-a7ce749679f0
DOI
10.1101/2020.09.15.297259
Open publication

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Inducible expression of human <i>C9ORF72</i> 36x G <sub>4</sub> C <sub>2</sub> hexanucleotide repeats is sufficient to cause RAN translation and rapid muscular atrophy in miceDOI 10.1101/2020.09.15.297259
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