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Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR

2 Jul 2019

Abstract excerpt

Abstract A GGGGCC hexanucleotide repeat expansion in intron 1 ofchromosome 9 open reading frame 72(C9ORF72) gene is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. Repeat-associated non-ATG translation of dipeptide repeat proteins (DPRs) contributes to the neuropathological features of c9FTD/ALS. Among the five DPRs, arginine-rich poly-PR are reported to be the...

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PMID: 31266945
DOI: 10.1038/s41467-019-10956-w

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