Unraveling the Role of RNA Mediated Toxicity of C9orf72 Repeats in C9-FTD/ALS
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Title
Unraveling the Role of RNA Mediated Toxicity of C9orf72 Repeats in C9-FTD/ALS
Authors
Keywords
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Journal
Frontiers in Neuroscience
Volume 11, Issue -, Pages -
Publisher
Frontiers Media SA
Online
2017-12-15
DOI
10.3389/fnins.2017.00711
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Note: Only part of the references are listed.- SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits
- (2017) Guillaume M. Hautbergue et al. Nature Communications
- The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD
- (2017) Brian D. Freibaum et al. Frontiers in Molecular Neuroscience
- There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALS
- (2016) Aaron D. Gitler et al. BRAIN RESEARCH
- C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles
- (2016) Kyung-Ha Lee et al. CELL
- Toxic PR Poly-Dipeptides Encoded by the C9orf72 Repeat Expansion Target LC Domain Polymers
- (2016) Yi Lin et al. CELL
- Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin‐2 to induce motor neuron dysfunction and cell death
- (2016) Chantal Sellier et al. EMBO JOURNAL
- The C9orf72 protein interacts with Rab1a and the ULK1 complex to regulate initiation of autophagy
- (2016) Christopher P Webster et al. EMBO JOURNAL
- The Glycine-Alanine Dipeptide Repeat from C9orf72 Hexanucleotide Expansions Forms Toxic Amyloids Possessing Cell-to-Cell Transmission Properties
- (2016) Yu-Jen Chang et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions
- (2016) Tiffany W. Todd et al. JOURNAL OF NEUROCHEMISTRY
- Development of Therapeutics for C9ORF72 ALS/FTD-Related Disorders
- (2016) Maria Sara Cipolat Mis et al. MOLECULAR NEUROBIOLOGY
- C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins
- (2016) Yong-Jie Zhang et al. NATURE NEUROSCIENCE
- Structural insight into C9orf72 hexanucleotide repeat expansions: Towards new therapeutic targets in FTD-ALS
- (2016) Vijay Kumar et al. NEUROCHEMISTRY INTERNATIONAL
- C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD
- (2016) Yuanjing Liu et al. NEURON
- Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs
- (2016) Jie Jiang et al. NEURON
- Bidirectional Transcriptional Inhibition as Therapy for ALS/FTD Caused by Repeat Expansion in C9orf72
- (2016) Jie Jiang et al. NEURON
- C9orf72 is required for proper macrophage and microglial function in mice
- (2016) J. G. ORourke et al. SCIENCE
- Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy
- (2015) Johnathan Cooper-Knock et al. ACTA NEUROPATHOLOGICA
- FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusions
- (2015) Dejun Yang et al. ACTA NEUROPATHOLOGICA
- Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity
- (2015) Zhouteng Tao et al. HUMAN MOLECULAR GENETICS
- Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS
- (2015) S. Rossi et al. JOURNAL OF CELL SCIENCE
- CRISPR-Cas9: a new and promising player in gene therapy
- (2015) Lu Xiao-Jie et al. JOURNAL OF MEDICAL GENETICS
- Drosha Inclusions Are New Components of Dipeptide-Repeat Protein Aggregates in FTLD-TDP and ALSC9orf72Expansion Cases
- (2015) Sílvia Porta et al. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
- C9orf72 expansions in frontotemporal dementia and amyotrophic lateral sclerosis
- (2015) Jonathan D Rohrer et al. LANCET NEUROLOGY
- The C9orf72 repeat size correlates with onset age of disease, DNA methylation and transcriptional downregulation of the promoter
- (2015) I Gijselinck et al. MOLECULAR PSYCHIATRY
- Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS
- (2015) Mercedes Prudencio et al. NATURE NEUROSCIENCE
- C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits
- (2015) J. Chew et al. SCIENCE
- C9ORF72 GGGGCC Expanded Repeats Produce Splicing Dysregulation which Correlates with Disease Severity in Amyotrophic Lateral Sclerosis
- (2015) Johnathan Cooper-Knock et al. PLoS One
- Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress
- (2014) Yong-Jie Zhang et al. ACTA NEUROPATHOLOGICA
- The widening spectrum of C9ORF72-related disease; genotype/phenotype correlations and potential modifiers of clinical phenotype
- (2014) Johnathan Cooper-Knock et al. ACTA NEUROPATHOLOGICA
- Mechanisms of toxicity in C9FTLD/ALS
- (2014) Tania F. Gendron et al. ACTA NEUROPATHOLOGICA
- C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration
- (2014) Stephanie May et al. ACTA NEUROPATHOLOGICA
- Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions
- (2014) Johnathan Cooper-Knock et al. BRAIN
- Therapeutic potentials of gene silencing by RNA interference: Principles, challenges, and new strategies
- (2014) Yan Deng et al. GENE
- C9orf72 nucleotide repeat structures initiate molecular cascades of disease
- (2014) Aaron R. Haeusler et al. NATURE
- Reduced C9orf72 protein levels in frontal cortex of amyotrophic lateral sclerosis and frontotemporal degeneration brain with the C9ORF72 hexanucleotide repeat expansion
- (2014) Adrian J. Waite et al. NEUROBIOLOGY OF AGING
- Unconventional features of C9ORF72 expanded repeat in amyotrophic lateral sclerosis and frontotemporal lobar degeneration
- (2014) Sabina Vatovec et al. NEUROBIOLOGY OF AGING
- Discovery of a Biomarker and Lead Small Molecules to Target r(GGGGCC)-Associated Defects in c9FTD/ALS
- (2014) Zhaoming Su et al. NEURON
- Antisense Proline-Arginine RAN Dipeptides Linked to C9ORF72-ALS/FTD Form Toxic Nuclear Aggregates that Initiate In Vitro and In Vivo Neuronal Death
- (2014) Xinmei Wen et al. NEURON
- Loss of nuclear TDP-43 in amyotrophic lateral sclerosis (ALS) causes altered expression of splicing machinery and widespread dysregulation of RNA splicing in motor neurones
- (2014) J. Robin Highley et al. NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY
- mRNP granules
- (2014) J Ross Buchan RNA Biology
- C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins
- (2014) S. Mizielinska et al. SCIENCE
- Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells
- (2014) I. Kwon et al. SCIENCE
- C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci
- (2013) Sarah Mizielinska et al. ACTA NEUROPATHOLOGICA
- Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS
- (2013) Tania F. Gendron et al. ACTA NEUROPATHOLOGICA
- hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations
- (2013) Kohji Mori et al. ACTA NEUROPATHOLOGICA
- Reduced C9orf72 gene expression in c9FTD/ALS is caused by histone trimethylation, an epigenetic event detectable in blood
- (2013) Veronique V. Belzil et al. ACTA NEUROPATHOLOGICA
- Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons
- (2013) Sandra Almeida et al. ACTA NEUROPATHOLOGICA
- Hypermethylation of the CpG Island Near the G4C2 Repeat in ALS with a C9orf72 Expansion
- (2013) Zhengrui Xi et al. AMERICAN JOURNAL OF HUMAN GENETICS
- The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs
- (2013) Timothy P. Levine et al. BIOINFORMATICS
- The mouse C9ORF72 ortholog is enriched in neurons known to degenerate in ALS and FTD
- (2013) Naoki Suzuki et al. NATURE NEUROSCIENCE
- Unconventional Translation of C9ORF72 GGGGCC Expansion Generates Insoluble Polypeptides Specific to c9FTD/ALS
- (2013) Peter E.A. Ash et al. NEURON
- RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention
- (2013) Christopher J. Donnelly et al. NEURON
- Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis
- (2013) Shuo-Chien Ling et al. NEURON
- RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia
- (2013) T. Zu et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration
- (2013) Z. Xu et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration
- (2013) C. Lagier-Tourenne et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS
- (2013) K. Mori et al. SCIENCE
- Targeting RNA Foci in iPSC-Derived Motor Neurons from ALS Patients with a C9ORF72 Repeat Expansion
- (2013) D. Sareen et al. Science Translational Medicine
- Current insights into the C9orf72 repeat expansion diseases of the FTLD/ALS spectrum
- (2013) Marc Cruts et al. TRENDS IN NEUROSCIENCES
- Hexanucleotide Repeats in ALS/FTD Form Length-Dependent RNA Foci, Sequester RNA Binding Proteins, and Are Neurotoxic
- (2013) Youn-Bok Lee et al. Cell Reports
- Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study
- (2012) Elisa Majounie et al. LANCET NEUROLOGY
- Cellular toxicity of expanded RNA repeats: focus on RNA foci
- (2011) Marzena Wojciechowska et al. HUMAN MOLECULAR GENETICS
- A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study
- (2011) Ilse Gijselinck et al. LANCET NEUROLOGY
- Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS
- (2011) Mariely DeJesus-Hernandez et al. NEURON
- A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD
- (2011) Alan E. Renton et al. NEURON
- Repeat expansion disease: progress and puzzles in disease pathogenesis
- (2010) Albert R. La Spada et al. NATURE REVIEWS GENETICS
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