Article

Ribosomal quality control factors inhibit repeat-associated non-AUG translation from GC-rich repeats

2023-06-07

Abstract excerpt

<h4>ABSTRACT</h4> A GGGGCC (G4C2) hexanucleotide repeat expansion in C9ORF72 causes amyotrophic lateral sclerosis and frontotemporal dementia (C9ALS/FTD), while a CGG trinucleotide repeat expansion in FMR1 leads to the neurodegenerative disorder Fragile X-associated tremor/ataxia syndrome (FXTAS). These GC-rich repeats form RNA secondary structures that support repeat-associated non-AUG (RAN) translation of tox...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: 7090a25b-2d1d-597b-a4c6-abfa7e7fba8a
DOI: 10.1101/2023.06.07.544135

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Ribosomal quality control factors inhibit repeat-associated non-AUG translation from GC-rich repeatsDOI 10.1101/2023.06.07.544135Read

Insufficiency of 40S ribosomal proteins, RPS26 and RPS25, negatively affects biosynthesis of polyglycine-containing proteins in fragile-X associated conditionsSemantic proximity Read

RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress responseSemantic proximity Read

CGG Repeat-Associated Non-AUG Translation Utilizes a Cap-Dependent Scanning Mechanism of Initiation to Produce Toxic Proteins.Semantic proximity Read

Repeat-associated non-ATG (RAN) translation.Semantic proximity Read

Repeat-associated non-AUG (RAN) translation and other molecular mechanisms in Fragile X Tremor Ataxia Syndrome.Semantic proximity Read

<i>In vivo</i> CGG repeat RNA binding protein capture identifies RAN translation modifiers and suppressors of repeat toxicitySemantic proximity Read

Unraveling the Role of RNA Mediated Toxicity of C9orf72 Repeats in C9-FTD/ALSSemantic proximity Read

Ribosome profiling reveals novel regulation of C9ORF72 GGGGCC repeat-containing RNA translationSemantic proximity Read

Reactivation of nonsense-mediated mRNA decay protects against C9orf72 dipeptide-repeat neurotoxicitySemantic proximity Read

Molecular mechanisms underlying nucleotide repeat expansion disorders.Semantic proximity Read

Cryptic intronic transcriptional initiation generates efficient endogenous mRNA templates for C9orf72-associated RAN translationSemantic proximity Read

RNA toxicity in non-coding repeat expansion disorders.Semantic proximity Read