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Dipeptide repeat proteins activate a heat shock response found in C9ORF72-ALS/FTLD patients

4 Jul 2018

Abstract excerpt

A hexanucleotide (GGGGCC) repeat expansion in C9ORF72 is the most common genetic contributor to amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Reduced expression of the C9ORF72 gene product has been proposed as a potential contributor to disease pathogenesis. Additionally, repetitive RNAs and dipeptide repeat proteins (DPRs), such as poly-GR, can be produced by this...

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PMID: 29973287
DOI: 10.1186/s40478-018-0555-8

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