FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusions
about
The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative MedicineMutant PFN1 causes ALS phenotypes and progressive motor neuron degeneration in mice by a gain of toxicity.C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Mouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal DementiaSRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits.Cerebellar c9RAN proteins associate with clinical and neuropathological characteristics of C9ORF72 repeat expansion carriers.Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS.Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity.The Glycine-Alanine Dipeptide Repeat from C9orf72 Hexanucleotide Expansions Forms Toxic Amyloids Possessing Cell-to-Cell Transmission PropertiesGain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAsOld versus New Mechanisms in the Pathogenesis of ALS.Evidence that C9ORF72 Dipeptide Repeat Proteins Associate with U2 snRNP to Cause Mis-splicing in ALS/FTD Patients.Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.RAN translation-What makes it run?Simple animal models for amyotrophic lateral sclerosis drug discovery.Review: Induced pluripotent stem cell models of frontotemporal dementia.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size.Sense-encoded poly-GR dipeptide repeat proteins correlate to neurodegeneration and uniquely co-localize with TDP-43 in dendrites of repeat-expanded C9orf72 amyotrophic lateral sclerosis.C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity.Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.Unraveling the Role of RNA Mediated Toxicity of C9orf72 Repeats in C9-FTD/ALS.Context-Dependent and Disease-Specific Diversity in Protein Interactions within Stress Granules.Synaptic dysfunction and altered excitability in C9ORF72 ALS/FTD.Downregulation of exosomal miR-204-5p and miR-632 as a biomarker for FTD: a GENFI study.Repeat-Associated Non-ATG (RAN) Translation in Fuchs' Endothelial Corneal Dystrophy.Poly-GR dipeptide repeat polymers correlate with neurodegeneration and Clinicopathological subtypes in C9ORF72-related brain disease
P2860
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P2860
FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusions
description
2015 nî lūn-bûn
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2015年の論文
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2015年学术文章
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2015年学术文章
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2015年学术文章
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2015年学术文章
@zh-my
2015年学术文章
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2015年學術文章
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name
FTD/ALS-associated poly(GR) pr ...... A) into cytoplasmic inclusions
@ast
FTD/ALS-associated poly(GR) pr ...... A) into cytoplasmic inclusions
@en
type
label
FTD/ALS-associated poly(GR) pr ...... A) into cytoplasmic inclusions
@ast
FTD/ALS-associated poly(GR) pr ...... A) into cytoplasmic inclusions
@en
prefLabel
FTD/ALS-associated poly(GR) pr ...... A) into cytoplasmic inclusions
@ast
FTD/ALS-associated poly(GR) pr ...... A) into cytoplasmic inclusions
@en
P2093
P2860
P1476
FTD/ALS-associated poly(GR) pr ...... A) into cytoplasmic inclusions
@en
P2093
Abbas Abdallah
Dejun Yang
Fen-Biao Gao
Sandra Almeida
Zhaodong Li
P2860
P2888
P304
P356
10.1007/S00401-015-1448-6
P50
P577
2015-06-02T00:00:00Z