Sense and antisense RNA are not toxic in Drosophila models of C9orf72-associated ALS/FTD. (Q48145508)

30 June 2019

title

Sense and antisense RNA are not toxic in Drosophila models of C9orf72-associated ALS/FTD. (English)

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30 June 2019

amyotrophic lateral sclerosis

main subject

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11

Linda Partridge

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PubMed publication ID

9 February 2018

Thomas G Moens

1

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Sarah Mizielinska

Sarah Mizielinska

2

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Teresa Niccoli

Teresa Niccoli

3

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Amanda Heslegrave

Amanda Heslegrave

9

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Sebastian Grönke

Sebastian Grönke

7

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Henrik Zetterberg

10

Henrik Zetterberg

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30 June 2019

12

Adrian M Isaacs

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30 June 2019

Charlotte Ridler

6

Charlotte E Ridler

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30 June 2019

Jacqueline Esser

8

Jacqueline Esser

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30 June 2019

author name string

Jamie S Mitchell

4

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30 June 2019

Annora Thoeng

5

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30 June 2019

publication date

29 January 2018

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30 June 2019

published in

Acta Neuropathologica

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30 June 2019

volume

135

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30 June 2019

issue

3

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30 June 2019

page(s)

445-457

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30 June 2019

A conditional tissue-specific transgene expression system using inducible GAL4.

cites work

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12 December 2020

CREB-binding protein modulates repeat instability in a Drosophila model for polyQ disease

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A protocol for imaging alternative splicing regulation in vivo using fluorescence reporters in transgenic mice

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Exploiting position effects and the gypsy retrovirus insulator to engineer precisely expressed transgenes

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Inhibition of GSK-3 ameliorates Abeta pathology in an adult-onset Drosophila model of Alzheimer's disease

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Perturbation of the Akt/Gsk3-β signalling pathway is common to Drosophila expressing expanded untranslated CAG, CUG and AUUCU repeat RNAs.

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Cellular toxicity of expanded RNA repeats: focus on RNA foci

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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

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12 December 2020

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

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Large C9orf72 hexanucleotide repeat expansions are seen in multiple neurodegenerative syndromes and are more frequent than expected in the UK population.

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

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Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis

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12 December 2020

Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort study

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

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

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12 December 2020

TMPyP4 porphyrin distorts RNA G-quadruplex structures of the disease-associated r(GGGGCC)n repeat of the C9orf72 gene and blocks interaction of RNA-binding proteins

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12 December 2020

C9orf72 nucleotide repeat structures initiate molecular cascades of disease.

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12 December 2020

Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Discovery of a biomarker and lead small molecules to target r(GGGGCC)-associated defects in c9FTD/ALS.

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12 December 2020

A census of human RNA-binding proteins

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12 December 2020

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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12 December 2020

C9orf72 expansions in frontotemporal dementia and amyotrophic lateral sclerosis

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12 December 2020

Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy.

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12 December 2020

C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits

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12 December 2020

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.

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12 December 2020

GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport

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12 December 2020

Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS.

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12 December 2020

Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS.

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12 December 2020

C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

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12 December 2020

Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice

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12 December 2020

GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function

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12 December 2020

Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD.

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12 December 2020

C9orf72 ablation causes immune dysregulation characterized by leukocyte expansion, autoantibody production, and glomerulonephropathy in mice.

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12 December 2020

Lithium Promotes Longevity through GSK3/NRF2-Dependent Hormesis

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12 December 2020

Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs

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12 December 2020

C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD.

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12 December 2020

The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease

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12 December 2020

The ALS/FTLD associated protein C9orf72 associates with SMCR8 and WDR41 to regulate the autophagy-lysosome pathway

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12 December 2020

Full ablation of C9orf72 in mice causes immune system-related pathology and neoplastic events but no motor neuron defects

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12 December 2020

Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease

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12 December 2020

Increased Glucose Transport into Neurons Rescues Aβ Toxicity in Drosophila

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12 December 2020

The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains.

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12 December 2020

Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106.

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12 December 2020

Genetic models of C9orf72: what is toxic?

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12 December 2020

In-depth clinico-pathological examination of RNA foci in a large cohort of C9ORF72 expansion carriers

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12 December 2020

RNA phase transitions in repeat expansion disorders

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12 December 2020

Major hnRNP proteins act as general TDP-43 functional modifiers both in Drosophila and human neuronal cells

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12 December 2020

SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits

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12 December 2020

G-quadruplex-binding small molecules ameliorate C9orf72 FTD/ALS pathology in vitro and in vivo.

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12 December 2020

10.1007/S00401-017-1798-3

Identifiers

DOI

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30 June 2019

PMC publication ID

6385858

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30 June 2019

PubMed publication ID

29380049

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30 June 2019