Article

Arginine-rich C9ORF72 ALS Proteins Stall Ribosomes in a Manner Distinct From a Canonical Ribosome-Associated Quality Control Substrate

2022-02-10

Abstract excerpt

<h4>ABSTRACT</h4> Hexanucleotide expansion mutations in C9ORF72 are a cause of familial amyotrophic lateral sclerosis. We previously reported that long arginine-rich dipeptide repeats (DPR), mimicking abnormal proteins expressed from the hexanucleotide expansion, caused translation stalling when expressed in cell culture models. Whether this stalling provides a mechanism of pathogenicity remains to be determined...

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Literature Corpus work: 2f577134-8db6-5c88-ba93-4aadfb7ecf9f
DOI: 10.1101/2022.02.09.479805

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