Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss.
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Pathogenesis of FUS-associated ALS and FTD: insights from rodent modelsCharacterization 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.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 degenerationTDP-43 and FUS en route from the nucleus to the cytoplasm.Functional analysis reveals that RBM10 mutations contribute to lung adenocarcinoma pathogenesis by deregulating splicingThe investigation of genetic and clinical features in Chinese patients with juvenile amyotrophic lateral sclerosis.Role of FET proteins in neurodegenerative disorders.Expanding Axonal Transcriptome Brings New Functions for Axonally Synthesized Proteins in Health and Disease.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.Motor Neuron Gene Therapy: Lessons from Spinal Muscular Atrophy for Amyotrophic Lateral Sclerosis.De novo design of RNA-binding proteins with a prion-like domain related to ALS/FTD proteinopathies.RNA binding proteins and the pathological cascade in ALS/FTD neurodegeneration.Molecular roles and function of circular RNAs in eukaryotic cells.To the end of the line: Axonal mRNA transport and local translation in health and neurodegenerative disease.RNA-binding proteins in neurodegeneration: mechanisms in aggregate.Humanized mutant FUS drives progressive motor neuron degeneration without aggregation in 'FUSDelta14' knockin mice.FUS Mutant Human Motoneurons Display Altered Transcriptome and microRNA Pathways with Implications for ALS Pathogenesis.Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice.A Regulatory Circuitry Between Gria2, miR-409, and miR-495 Is Affected by ALS FUS Mutation in ESC-Derived Motor Neurons.Protein-RNA Networks Regulated by Normal and ALS-Associated Mutant HNRNPA2B1 in the Nervous System.RNA buffers the phase separation behavior of prion-like RNA binding proteins.The Role of Post-Translational Modifications on Prion-Like Aggregation and Liquid-Phase Separation of FUS.Linking hnRNP Function to ALS and FTD Pathology.Nuclear egress of TDP-43 and FUS occurs independently of Exportin-1/CRM1.Importance of Functional Loss of FUS in FTLD/ALS.A complex of C9ORF72 and p62 uses arginine methylation to eliminate stress granules by autophagyAmyotrophic Lateral Sclerosis associated FUS mutation shortens mitochondria and induces neurotoxicityAxonal Transport, Phase-Separated Compartments, and Neuron Mechanics - A New Approach to Investigate Neurodegenerative Diseasesgenetically interacts with the ALS-associated orthologue and mediates its toxicity
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P2860
Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss.
description
2016 nî lūn-bûn
@nan
2016年の論文
@ja
2016年論文
@yue
2016年論文
@zh-hant
2016年論文
@zh-hk
2016年論文
@zh-mo
2016年論文
@zh-tw
2016年论文
@wuu
2016年论文
@zh
2016年论文
@zh-cn
name
Toxic gain of function from mu ...... autonomous motor neuron loss.
@en
type
label
Toxic gain of function from mu ...... autonomous motor neuron loss.
@en
altLabel
Toxic gain of function from mu ...... l autonomous motor neuron loss
@en
prefLabel
Toxic gain of function from mu ...... autonomous motor neuron loss.
@en
P2093
P2860
P50
P356
P1433
P1476
Toxic gain of function from mu ...... autonomous motor neuron loss.
@en
P2093
Anke Witting
Hajer El Oussini
Jelena Scekic-Zahirovic
Jinsong Qiu
Jérome Sinniger
Kevin Drenner
Luc Dupuis
Marina Wagner
Mélanie Jambeau
P2860
P304
P356
10.15252/EMBJ.201592559
P407
P50
P577
2016-03-07T00:00:00Z