A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism
Published 2018 View Full Article
- Home
- Publications
- Publication Search
- Publication Details
Title
A zebrafish model for C9orf72 ALS reveals RNA toxicity as a pathogenic mechanism
Authors
Keywords
ALS, C9orf72, DPR, RNA toxicity, Pur-alpha, p62, Zebrafish
Journal
ACTA NEUROPATHOLOGICA
Volume 135, Issue 3, Pages 427-443
Publisher
Springer Nature
Online
2018-01-04
DOI
10.1007/s00401-017-1796-5
References
Ask authors/readers for more resources
Related references
Note: Only part of the references are listed.- Glycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegeneration
- (2017) Yu Ohki et al. Molecular Neurodegeneration
- The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD
- (2017) Brian D. Freibaum et al. Frontiers in Molecular Neuroscience
- Full ablation of C9orf72 in mice causes immune system-related pathology and neoplastic events but no motor neuron defects
- (2016) Emma Sudria-Lopez et al. ACTA NEUROPATHOLOGICA
- There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALS
- (2016) Aaron D. Gitler et al. BRAIN RESEARCH
- C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins
- (2016) Yong-Jie Zhang et al. NATURE NEUROSCIENCE
- The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease
- (2016) Aaron R. Haeusler et al. NATURE REVIEWS NEUROSCIENCE
- C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD
- (2016) Yuanjing Liu et al. NEURON
- C9orf72 is required for proper macrophage and microglial function in mice
- (2016) J. G. ORourke et al. SCIENCE
- C9orf72 Hexanucleotide Expansions Are Associated with Altered Endoplasmic Reticulum Calcium Homeostasis and Stress Granule Formation in Induced Pluripotent Stem Cell-Derived Neurons from Patients with Amyotrophic Lateral Sclerosis and Frontotemporal Demen
- (2016) Ruxandra Dafinca et al. STEM CELLS
- The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains
- (2016) Erin G Conlon et al. eLife
- Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD
- (2016) Steven Boeynaems et al. Scientific Reports
- C9orf72 ablation causes immune dysregulation characterized by leukocyte expansion, autoantibody production and glomerulonephropathy in mice
- (2016) Amanda Atanasio et al. Scientific Reports
- 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
- Cerebellar c9RAN proteins associate with clinical and neuropathological characteristics of C9ORF72 repeat expansion carriers
- (2015) Tania F. Gendron et al. ACTA NEUROPATHOLOGICA
- C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits
- (2015) Max Koppers et al. ANNALS OF NEUROLOGY
- 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
- Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions
- (2014) Johnathan Cooper-Knock et al. BRAIN
- Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS
- (2014) Mai Yamakawa et al. HUMAN MOLECULAR GENETICS
- p62 Plays a Protective Role in the Autophagic Degradation of Polyglutamine Protein Oligomers in Polyglutamine Disease Model Flies
- (2014) Yuji Saitoh et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- C9orf72 nucleotide repeat structures initiate molecular cascades of disease
- (2014) Aaron R. Haeusler et al. NATURE
- C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins
- (2014) S. Mizielinska et al. SCIENCE
- Homozygosity for the C9orf72 GGGGCC repeat expansion in frontotemporal dementia
- (2013) Pietro Fratta et al. ACTA NEUROPATHOLOGICA
- C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci
- (2013) Sarah Mizielinska 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
- Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology
- (2013) Elisa Teyssou 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
- Loss of function of C9orf72 causes motor deficits in a zebrafish model of Amyotrophic Lateral Sclerosis
- (2013) Sorana Ciura et al. ANNALS OF NEUROLOGY
- Association between repeat sizes and clinical and pathological characteristics in carriers of C9ORF72 repeat expansions (Xpansize-72): a cross-sectional cohort study
- (2013) Marka van Blitterswijk et al. LANCET NEUROLOGY
- State of play in amyotrophic lateral sclerosis genetics
- (2013) Alan E Renton et al. NATURE NEUROSCIENCE
- The changing scene of amyotrophic lateral sclerosis
- (2013) Wim Robberecht et al. NATURE REVIEWS NEUROSCIENCE
- C9ORF72 hexanucleotide expansions of 20-22 repeats are associated with frontotemporal deterioration
- (2013) E. Gomez-Tortosa et al. NEUROLOGY
- RNA Toxicity from the ALS/FTD C9ORF72 Expansion Is Mitigated by Antisense Intervention
- (2013) Christopher J. Donnelly et al. NEURON
- Deletion of C9ORF72 Results in Motor Neuron Degeneration and Stress Sensitivity in C. elegans
- (2013) Martine Therrien et al. PLoS One
- 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
- 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
- The myotonic dystrophies: molecular, clinical, and therapeutic challenges
- (2012) Bjarne Udd et al. LANCET NEUROLOGY
- EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans
- (2012) Annelies Van Hoecke et al. NATURE MEDICINE
- TRAF6 and p62 inhibit amyloid β-induced neuronal death through p75 neurotrophin receptor
- (2012) Thangiah Geetha et al. NEUROCHEMISTRY INTERNATIONAL
- 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
- Regulation of TDP-43 aggregation by phosphorylation andp62/SQSTM1
- (2010) Owen A. Brady et al. JOURNAL OF NEUROCHEMISTRY
- Progranulin is Neurotrophic In Vivo and Protects against a Mutant TDP-43 Induced Axonopathy
- (2010) Angela S. Laird et al. PLoS One
Discover Peeref hubs
Discuss science. Find collaborators. Network.
Join a conversationAsk a Question. Answer a Question.
Quickly pose questions to the entire community. Debate answers and get clarity on the most important issues facing researchers.
Get Started