ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import - Wikidata - awgldk - TriplyDB

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

http://www.wikidata.org/entity/Q24630100

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FUS stimulates microRNA biogenesis by facilitating co-transcriptional Drosha recruitment Altered ribostasis: RNA-protein granules in degenerative disorders Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis Nuclear transport, oxidative stress, and neurodegeneration Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS Inside out: the role of nucleocytoplasmic transport in ALS and FTLD Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis Dysregulated axonal RNA translation in amyotrophic lateral sclerosis Prion-like domains as epigenetic regulators, scaffolds for subcellular organization, and drivers of neurodegenerative disease FUS-mediated regulation of alternative RNA processing in neurons: insights from global transcriptome analysis Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS?Brain-Specific Cytoskeletal Damage Markers in Cerebrospinal Fluid: Is There a Common Pattern between Amyotrophic Lateral Sclerosis and Primary Progressive Multiple Sclerosis?Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis The function of RNA-binding proteins at the synapse: implications for neurodegeneration Redox regulation in amyotrophic lateral sclerosis Cytoplasmic sequestration of FUS/TLS associated with ALS alters histone marks through loss of nuclear protein arginine methyltransferase 1.Early lethality and neuronal proteinopathy in mice expressing cytoplasm-targeted FUS that lacks the RNA recognition motif.FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration Structural and energetic basis of ALS-causing mutations in the atypical proline–tyrosine nuclear localization signal of the Fused in Sarcoma protein (FUS)Structural and molecular insights into the mechanism of action of human angiogenin-ALS variants in neurons TDP-43 aggregation in neurodegeneration: are stress granules the key?A Case for Microtubule Vulnerability in Amyotrophic Lateral Sclerosis: Altered Dynamics During Disease TDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.Emerging mechanisms of molecular pathology in ALS Engineering enhanced protein disaggregases for neurodegenerative disease Drosophila as an In Vivo Model for Human Neurodegenerative Disease Transportin1: a marker of FTLD-FUS Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations FUS-NLS/Transportin 1 complex structure provides insights into the nuclear targeting mechanism of FUS and the implications in ALS Identification of genetic modifiers of TDP-43 neurotoxicity in Drosophila FUS Interacts with HSP60 to Promote Mitochondrial Damage EWS and FUS bind a subset of transcribed genes encoding proteins enriched in RNA regulatory functions Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis Are aberrant phase transitions a driver of cellular aging?Mutational analysis of FUS gene and its structural and functional role in amyotrophic lateral sclerosis 6.Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion.Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasm

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ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

http://www.wikidata.org/entity/Q24630100

description

2010 nî lūn-bûn

@nan

2010 թուականի Օգոստոսին հրատարակուած գիտական յօդուած

@hyw

2010 թվականի օգոստոսին հրատարակված գիտական հոդված

@hy

2010年の論文

@ja

2010年論文

@yue

2010年論文

@zh-hant

2010年論文

@zh-hk

2010年論文

@zh-mo

2010年論文

@zh-tw

2010年论文

@wuu

name

ALS-associated fused in sarcom ...... portin-mediated nuclear import

@ast

ALS-associated fused in sarcom ...... portin-mediated nuclear import

@en

ALS-associated fused in sarcom ...... portin-mediated nuclear import

@nl

type

label

ALS-associated fused in sarcom ...... portin-mediated nuclear import

@ast

ALS-associated fused in sarcom ...... portin-mediated nuclear import

@en

ALS-associated fused in sarcom ...... portin-mediated nuclear import

@nl

prefLabel

ALS-associated fused in sarcom ...... portin-mediated nuclear import

@ast

ALS-associated fused in sarcom ...... portin-mediated nuclear import

@en

ALS-associated fused in sarcom ...... portin-mediated nuclear import

@nl

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The EMBO Journal

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ALS-associated fused in sarcom ...... portin-mediated nuclear import

@en

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Alexander Hruscha

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

Bettina Schmid

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

Dorothee Dormann

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

Eva Bentmann

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

Ian R A Mackenzie

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

Ingeborg Fischer

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

Manuel E Than

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

Ramona Rodde

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

P2860

Transportins 1 and 2 are redundant nuclear import factors for hnRNP A1 and HuR

TLS-ERG leukemia fusion protein inhibits RNA splicing mediated by serine-arginine proteins

Rules for nuclear localization sequence recognition by karyopherin beta 2

Transportin2 functions as importin and mediates nuclear import of HuR

Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease

A new subtype of frontotemporal lobar degeneration with FUS pathology

Structure-based design of a pathway-specific nuclear import inhibitor

Structural basis for substrate recognition and dissociation by human transportin 1

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

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2841-57

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scholarly article

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3544474

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10.1038/EMBOJ.2010.143

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16

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29

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Christian Haass

Manuela Neumann

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2010-08-18T00:00:00Z

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45097584

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20606625

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amyotrophic lateral sclerosis

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2924641

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