Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity (Q38921570)

7 September 2017

title

Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity (English)

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7 September 2017

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Zhouteng Tao

1

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7 September 2017

Hongfeng Wang

2

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7 September 2017

Qin Xia

3

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7 September 2017

Ke Li

4

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7 September 2017

Kai Li

5

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7 September 2017

Xiaogang Jiang

6

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7 September 2017

Guoqiang Xu

7

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7 September 2017

Guanghui Wang

8

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7 September 2017

Zheng Ying

9

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7 September 2017

publication date

9 January 2015

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7 September 2017

number of pages

16

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based on heuristic

inferred from page(s)

published in

Human Molecular Genetics

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7 September 2017

volume

24

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7 September 2017

issue

9

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7 September 2017

page(s)

2426-2441

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Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

7 September 2017

cites work

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A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

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Repeat expansion in C9ORF72 in Alzheimer's disease

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C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies

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C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

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Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans

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RNA toxicity in human disease and animal models: from the uncovering of a new mechanism to the development of promising therapies

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C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci.

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RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention

21 January 2018

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

21 January 2018

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Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration

21 January 2018

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Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic

21 January 2018

1 reference

RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia

21 January 2018

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Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration

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C9orf72 nucleotide repeat structures initiate molecular cascades of disease.

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Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons

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hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations

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Non-ATG-initiated translation directed by microsatellite expansions

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Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS

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The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.

21 January 2018

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CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome

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Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins

21 January 2018

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Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72.

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C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins

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Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells

21 January 2018

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21 January 2018

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The ubiquitin proteasome system as a potential target for the treatment of neurodegenerative diseases

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The roles of intracellular protein-degradation pathways in neurodegeneration

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B23 acts as a nucleolar stress sensor and promotes cell survival through its dynamic interaction with hnRNPU and hnRNPA1.

21 January 2018

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Nucleolar stress is an early response to myocardial damage involving nucleolar proteins nucleostemin and nucleophosmin

21 January 2018

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Ribosomal protein S9 is a novel B23/NPM-binding protein required for normal cell proliferation

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A new PICTure of nucleolar stress.

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Stress granules as crucibles of ALS pathogenesis.

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Mechanisms of toxicity in C9FTLD/ALS.

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21 January 2018

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21 January 2018

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Intracellular localization of homopolymeric amino acid-containing proteins expressed in mammalian cells

21 January 2018

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Aggregate formation prevents dTDP-43 neurotoxicity in the Drosophila melanogaster eye.

21 January 2018

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Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

21 January 2018

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Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

21 January 2018

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Directed proteomic analysis of the human nucleolus

21 January 2018

1 reference

Functional proteomic analysis of human nucleolus

21 January 2018

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How Parkinson's disease meets nucleolar stress

21 January 2018

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Nucleolar dysfunction in Huntington's disease

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The nucleolus under stress

21 January 2018

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RNA granules: post-transcriptional and epigenetic modulators of gene expression

21 January 2018

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An ortholog of the Ro autoantigen functions in 23S rRNA maturation in D. radiodurans

21 January 2018

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An RNA degradation machine sculpted by Ro autoantigen and noncoding RNA

21 January 2018

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Degradation of RNA in bacteria: comparison of mRNA and stable RNA

21 January 2018

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Mammalian stress granules and processing bodies

21 January 2018

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Gp78, an ER associated E3, promotes SOD1 and ataxin-3 degradation.

21 January 2018

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p45, an ATPase subunit of the 19S proteasome, targets the polyglutamine disease protein ataxin-3 to the proteasome.

21 January 2018

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Ataxin-3 regulates aggresome formation of copper-zinc superoxide dismutase (SOD1) by editing K63-linked polyubiquitin chains

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p62/sequestosome 1 regulates aggresome formation of pathogenic ataxin-3 with expanded polyglutamine

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The endoplasmic reticulum (ER)-associated degradation system regulates aggregation and degradation of mutant neuroserpin.

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21 January 2018

Identifiers

DOI

10.1093/HMG/DDV005

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7 September 2017

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