Toxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export. - Wikidata - wikimedia - TriplyDB

Toxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export.

Toxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export.

http://www.wikidata.org/entity/Q37658935

about

TDP-43 and FUS en route from the nucleus to the cytoplasm.Evidence that C9ORF72 Dipeptide Repeat Proteins Associate with U2 snRNP to Cause Mis-splicing in ALS/FTD Patients.Mutant Huntingtin Disrupts the Nuclear Pore Complex.The nucleus: keeping it together by keeping it apart.The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.The Enigmatic Role of C9ORF72 in Autophagy Failure to Deliver and Translate-New Insights into RNA Dysregulation in ALS.RNA biology of disease-associated microsatellite repeat expansions.A PR plug for the nuclear pore in amyotrophic lateral sclerosis.Intrinsic Disorder in Proteins with Pathogenic Repeat Expansions.Lost in Transportation: Nucleocytoplasmic Transport Defects in ALS and Other Neurodegenerative Diseases.Structure of FUS Protein Fibrils and Its Relevance to Self-Assembly and Phase Separation of Low-Complexity Domains.MIF inhibits the formation and toxicity of misfolded SOD1 amyloid aggregates: implications for familial ALS.C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2α phosphorylation.Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.Repeat-associated non-AUG (RAN) translation and other molecular mechanisms in Fragile X Tremor Ataxia Syndrome.Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells.TDP43 and RNA instability in amyotrophic lateral sclerosis.Cross-β polymerization and hydrogel formation by low-complexity sequence proteins.Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport.DNA plasticity and damage in amyotrophic lateral sclerosis.CRISPR-Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity.Stress Granule Assembly Disrupts Nucleocytoplasmic Transport.TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia.Structural and functional analysis of mRNA export regulation by the nuclear pore complex.Interactome analyses revealed that the U1 snRNP machinery overlaps extensively with the RNAP II machinery and contains multiple ALS/SMA-causative proteins.

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Toxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export.

http://www.wikidata.org/entity/Q37658935

description

article científic

@ca

article scientifique

@fr

articolo scientifico

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artigo científico

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bilimsel makale

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scientific article published on 09 January 2017

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vedecký článok

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vetenskaplig artikel

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videnskabelig artikel

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vědecký článek

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name

Toxic PRn poly-dipeptides enco ...... ock nuclear import and export.

@en

Toxic PRn poly-dipeptides enco ...... ock nuclear import and export.

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type

label

Toxic PRn poly-dipeptides enco ...... ock nuclear import and export.

@en

Toxic PRn poly-dipeptides enco ...... ock nuclear import and export.

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prefLabel

Toxic PRn poly-dipeptides enco ...... ock nuclear import and export.

@en

Toxic PRn poly-dipeptides enco ...... ock nuclear import and export.

@nl

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PNAS.1620293114

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Proceedings of the National Academy of Sciences of the United States of America

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Toxic PRn poly-dipeptides enco ...... ock nuclear import and export.

@en

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Eiichiro Mori

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

Joseph G Gall

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

Kevin Y Shi

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Leeju C Wu

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Masato Kato

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Steven L McKnight

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Yi Lin

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Yonghao Yu

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

Zehra F Nizami

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

P2860

Facilitated aggregation of FG nucleoporins under molecular crowding conditions

The peptide repeat domain of nucleoporin Nup98 functions as a docking site in transport across the nuclear pore complex

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

Interaction between NTF2 and xFxFG-containing nucleoporins is required to mediate nuclear import of RanGDP

Minimal nuclear pore complexes define FG repeat domains essential for transport.

Binding dynamics of structural nucleoporins govern nuclear pore complex permeability and may mediate channel gating

Natively unfolded nucleoporins gate protein diffusion across the nuclear pore complex.

Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogels

Nuclear protein import in permeabilized mammalian cells requires soluble cytoplasmic factors

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scholarly article

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10.1073/PNAS.1620293114

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114

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2017-01-09T00:00:00Z

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