Article

Antisense, but not sense, repeat expanded RNAs activate PKR/eIF2α-dependent integrated stress response in C9orf72 FTD/ALS

2022-06-06

Abstract excerpt

GGGGCC (G 4 C 2 ) hexanucleotide repeat expansion in the C9orf72 gene is the most common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). The repeat is bidirectionally transcribed and confers gain of toxicity. However, the underlying toxic species is debated, and it is not clear whether antisense CCCCGG (C 4 G 2 ) repeat expanded RNAs contribute to disease pathogenesis. O...

Topics

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

Identifiers and source

Literature Corpus work: a6f829b6-1f30-55e7-9058-ffd9ad88859e
DOI: 10.1101/2022.06.06.495030

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Antisense, but not sense, repeat expanded RNAs activate PKR/eIF2α-dependent integrated stress response in C9orf72 FTD/ALSDOI 10.1101/2022.06.06.495030Read

Antisense, but not sense, repeat expanded RNAs activate PKR/eIF2α-dependent ISR in C9ORF72 FTD/ALSSemantic proximity Read

C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2α phosphorylationSemantic proximity Read

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

RNA-mediated toxicity in C9orf72 ALS and FTDSemantic proximity Read

There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALS.Semantic proximity Read

RAN proteins and RNA foci from antisense transcripts in <i>C9ORF72</i> ALS and frontotemporal dementiaSemantic proximity Read

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

Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALSSemantic proximity Read

C9orf72; abnormal RNA expression is the key.Semantic proximity Read

The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTDSemantic proximity Read

Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansionsSemantic proximity Read

Distinct neurological disorders with C9orf72 mutations: genetics, pathogenesis, and therapy.Semantic proximity Read