(Q36246294)

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Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death

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Europe PubMed Central

PMC publication ID

13 August 2017

Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death (English)

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Europe PubMed Central

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13 August 2017

amyotrophic lateral sclerosis

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Karthik Krishnamurthy

4

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:25521377%20AND%20SRC:MED&resulttype=core&format=json

11 June 2020

Neil A. Shneider

8

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11 June 2020

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Xinmei Wen

1

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11 June 2020

Wenzhi Tan

2

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11 June 2020

Thomas Westergard

3

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11 June 2020

Shashirekha S Markandaiah

5

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11 June 2020

Yingxiao Shi

6

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11 June 2020

Shaoyu Lin

7

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11 June 2020

John Monaghan

9

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11 June 2020

Udai B Pandey

10

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11 June 2020

Piera Pasinelli

11

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11 June 2020

Justin K Ichida

12

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11 June 2020

Davide Trotti

13

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11 June 2020

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1 December 2014

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13 August 2017

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11 June 2020

84

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11 June 2020

6

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11 June 2020

1213-1225

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11 June 2020

describes a project that uses

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11 June 2022

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C9orf72 amyotrophic lateral sclerosis and frontotemporal dementia: gain or loss of function?

1 reference

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1 October 2017

Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

1 reference

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1 October 2017

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

1 reference

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1 October 2017

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

1 reference

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1 October 2017

C9orf72 nucleotide repeat structures initiate molecular cascades of disease.

1 reference

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1 October 2017

The C9ORF72 expansion mutation: gene structure, phenotypic and diagnostic issues.

1 reference

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1 October 2017

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

1 reference

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1 October 2017

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.

1 reference

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1 October 2017

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

1 reference

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1 October 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4632245

1 October 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4632245

1 October 2017

Clinical review. Frontotemporal dementia

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4632245

1 October 2017

Hypermethylation of the CpG island near the G4C2 repeat in ALS with a C9orf72 expansion

1 reference

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1 October 2017

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

1 reference

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1 October 2017

Cognitive changes predict functional decline in ALS: a population-based longitudinal study

1 reference

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1 October 2017

The disease-associated r(GGGGCC)n repeat from the C9orf72 gene forms tract length-dependent uni- and multimolecular RNA G-quadruplex structures

1 reference

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1 October 2017

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

1 reference

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1 October 2017

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

1 reference

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1 October 2017

RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations

1 reference

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1 October 2017

Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype

1 reference

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1 October 2017

Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study

1 reference

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1 October 2017

Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study

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Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons

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1 October 2017

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

1 reference

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1 October 2017

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

1 reference

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1 October 2017

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1 reference

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A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.

1 reference

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1 reference

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Amyotrophic lateral sclerosis

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https://pubmed.ncbi.nlm.nih.gov/25521377

12 December 2020

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10.1016/J.NEURON.2014.12.010

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11 June 2020

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24 November 2022

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11 June 2020

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11 June 2020

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