ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function. - Wikidata - awgldk - TriplyDB

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.

http://www.wikidata.org/entity/Q36548825

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Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis Pathogenesis of FUS-associated ALS and FTD: insights from rodent models The bowel and beyond: the enteric nervous system in neurological disorders Monitoring peripheral nerve degeneration in ALS by label-free stimulated Raman scattering imaging.Characterization of genetic loss-of-function of Fus in zebrafish.Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.ALS-linked FUS exerts a gain of toxic function involving aberrant p38 MAPK activation.CRISPR/Cas9-mediated targeted gene correction in amyotrophic lateral sclerosis patient iPSCs.Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis.Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice.RNA metabolism in neurodegenerative disease.FUScinating insights into motor neuron degeneration TDP-43 and FUS en route from the nucleus to the cytoplasm.Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses.Changes in the Expression of FUS/TLS in Spinal Cords of SOD1 G93A Transgenic Mice and Correlation with Motor-Neuron Degeneration.Neuromuscular Junction Impairment in Amyotrophic Lateral Sclerosis: Reassessing the Role of Acetylcholinesterase.Gamma motor neurons survive and exacerbate alpha motor neuron degeneration in ALS.FUS affects circular RNA expression in murine embryonic stem cell-derived motor neurons.Modelling FUS Mislocalisation in an In Vitro Model of Innervated Human Muscle.Stress granules at the intersection of autophagy and ALS.Aggregation of FET Proteins as a Pathological Change in Amyotrophic Lateral Sclerosis.Role of FET proteins in neurodegenerative disorders.TDP-43/FUS in motor neuron disease: Complexity and challenges.Spatially and temporally regulating translation via mRNA-binding proteins in cellular and neuronal function.New routes in frontotemporal dementia drug discovery.DOK7 gene therapy enhances motor activity and life span in ALS model mice.HDAC6 inhibition reverses axonal transport defects in motor neurons derived from FUS-ALS patients.Genetic mutations in RNA-binding proteins and their roles in ALS.Phosphorylation of the FUS low-complexity domain disrupts phase separation, aggregation, and toxicity.Dendritic Homeostasis Disruption in a Novel Frontotemporal Dementia Mouse Model Expressing Cytoplasmic Fused in Sarcoma.Nucleic acid binding proteins affect the subcellular distribution of phosphorothioate antisense oligonucleotides.Converging Mechanisms of p53 Activation Drive Motor Neuron Degeneration in Spinal Muscular Atrophy.De novo design of RNA-binding proteins with a prion-like domain related to ALS/FTD proteinopathies.Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice.RNA binding proteins and the pathological cascade in ALS/FTD neurodegeneration.RNA-binding proteins in neurodegeneration: mechanisms in aggregate.Humanized mutant FUS drives progressive motor neuron degeneration without aggregation in 'FUSDelta14' knockin mice.Liquidizing FUS via prion-like domain phosphorylation.Role and Therapeutic Potential of Astrocytes in Amyotrophic Lateral Sclerosis.Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice.

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ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.

http://www.wikidata.org/entity/Q36548825

description

2016 nî lūn-bûn

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ALS-associated mutant FUS indu ...... hrough toxic gain of function.

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ALS-associated mutant FUS indu ...... hrough toxic gain of function.

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ALS-associated mutant FUS indu ...... hrough toxic gain of function.

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ALS-associated mutant FUS indu ...... hrough toxic gain of function.

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ALS-associated mutant FUS indu ...... hrough toxic gain of function.

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Nature Communications

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ALS-associated mutant FUS indu ...... hrough toxic gain of function.

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Aarti Sharma

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

Adriana Nemes

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Alexander K Lyashchenko

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George Z Mentis

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Juan Carlos Tapia

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

Lei Lu

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

Margot Elmaleh

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

Monica Mendelsohn

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Neil A Shneider

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Sara Ebrahimi Nasrabady

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P2860

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

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

A developmental switch in the response of DRG neurons to ETS transcription factor signaling

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

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

Proteomic analysis of NMDA receptor-adhesion protein signaling complexes

Early and selective loss of neuromuscular synapse subtypes with low sprouting competence in motoneuron diseases

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6

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293015007

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26842965

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amyotrophic lateral sclerosis

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4742863

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