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

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

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

http://www.wikidata.org/entity/Q36246294

about

Inside out: the role of nucleocytoplasmic transport in ALS and FTLD There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALS Cell-based screening: extracting meaning from complex data.Emerging mechanisms of molecular pathology in ALS G-quadruplexes: Emerging roles in neurodegenerative diseases and the non-coding transcriptome Drosophila as an In Vivo Model for Human Neurodegenerative Disease Glycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegeneration Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell death A C9ORF72/SMCR8-containing complex regulates ULK1 and plays a dual role in autophagy Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition Toxic PR Poly-Dipeptides Encoded by the C9orf72 Repeat Expansion Target LC Domain Polymers.Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD.C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Modelling amyotrophic lateral sclerosis: progress and possibilities Mouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal Dementia GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport.Linking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis.Probing disorders of the nervous system using reprogramming approaches.Dipeptide repeat protein inclusions are rare in the spinal cord and almost absent from motor neurons in C9ORF72 mutant amyotrophic lateral sclerosis and are unlikely to cause their degeneration.MBNL Sequestration by Toxic RNAs and RNA Misprocessing in the Myotonic Dystrophy Brain FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusions Cerebellar c9RAN proteins associate with clinical and neuropathological characteristics of C9ORF72 repeat expansion carriers.Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing 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.C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits.Retention of hexanucleotide repeat-containing intron in C9orf72 mRNA: implications for the pathogenesis of ALS/FTD The Glycine-Alanine Dipeptide Repeat from C9orf72 Hexanucleotide Expansions Forms Toxic Amyloids Possessing Cell-to-Cell Transmission Properties Concise Review: Progress and Challenges in Using Human Stem Cells for Biological and Therapeutics Discovery: Neuropsychiatric Disorders.A low absolute number of expanded transcripts is involved in myotonic dystrophy type 1 manifestation in muscle.Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice Distinct brain transcriptome profiles in C9orf72-associated and sporadic ALS.Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation ALS mutant SOD1 interacts with G3BP1 and affects stress granule dynamics Forward Genetic Screen in Caenorhabditis elegans Suggests F57A10.2 and acp-4 As Suppressors of C9ORF72 Related Phenotypes.C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins.

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P2860

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

http://www.wikidata.org/entity/Q36246294

description

2014 nî lūn-bûn

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2014年の論文

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2014年論文

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2014年論文

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2014年論文

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2014年論文

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2014年論文

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2014年论文

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2014年论文

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2014年论文

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name

Antisense proline-arginine RAN ...... ro and in vivo neuronal death.

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Antisense proline-arginine RAN ...... ro and in vivo neuronal death.

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type

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Antisense proline-arginine RAN ...... ro and in vivo neuronal death.

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Antisense proline-arginine RAN ...... ro and in vivo neuronal death.

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prefLabel

Antisense proline-arginine RAN ...... ro and in vivo neuronal death.

@ast

Antisense proline-arginine RAN ...... ro and in vivo neuronal death.

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J.NEURON.2014.12.010

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Antisense proline-arginine RAN ...... ro and in vivo neuronal death.

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Davide Trotti

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John Monaghan

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Justin K Ichida

http://www.w3.org/2001/XMLSchema#string

Piera Pasinelli

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Shaoyu Lin

http://www.w3.org/2001/XMLSchema#string

Shashirekha S Markandaiah

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Thomas Westergard

http://www.w3.org/2001/XMLSchema#string

Udai B Pandey

http://www.w3.org/2001/XMLSchema#string

Wenzhi Tan

http://www.w3.org/2001/XMLSchema#string

Xinmei Wen

http://www.w3.org/2001/XMLSchema#string

P2860

Ataxin-2 interacts with the DEAD/H-box RNA helicase DDX6 and interferes with P-bodies and stress granules

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

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

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

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

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

Clinical review. Frontotemporal dementia

HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS

C9orf72 nucleotide repeat structures initiate molecular cascades of disease.

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

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1213-1225

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6

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84

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Neil A. Shneider

Karthik Krishnamurthy

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269692065

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amyotrophic lateral sclerosis

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