ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism.
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From animal models to human disease: a genetic approach for personalized medicine in ALSThe function of RNA-binding proteins at the synapse: implications for neurodegenerationMutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegansStructural and energetic basis of ALS-causing mutations in the atypical proline–tyrosine nuclear localization signal of the Fused in Sarcoma protein (FUS)Pathogenesis of FUS-associated ALS and FTD: insights from rodent modelsOxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutationsFUS Interacts with HSP60 to Promote Mitochondrial DamageReduced expression of BTBD10, an Akt activator, leads to motor neuron death.Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion.Recruitment into stress granules prevents irreversible aggregation of FUS protein mislocalized to the cytoplasmThe ALS gene FUS regulates synaptic transmission at the Drosophila neuromuscular junction.Multistep process of FUS aggregation in the cell cytoplasm involves RNA-dependent and RNA-independent mechanisms.Activity-dependent FUS dysregulation disrupts synaptic homeostasisCharacterization of FUS mutations in amyotrophic lateral sclerosis using RNA-Seq.ALS-associated FUS mutations result in compromised FUS alternative splicing and autoregulation.Exome sequencing identifies FUS mutations as a cause of essential tremor.Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats.ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule Function.Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis.Probing amyloid protein aggregation with optical superresolution methods: from the test tube to models of disease.Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice.Review: Axon pathology in age-related neurodegenerative disorders.Protein aggregation in amyotrophic lateral sclerosis.Discovery of protein-RNA networks.The role of FUS gene variants in neurodegenerative diseases.Worming forward: amyotrophic lateral sclerosis toxicity mechanisms and genetic interactions in Caenorhabditis elegans.Use of Caenorhabditis elegans as a model to study Alzheimer's disease and other neurodegenerative diseases.Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS.Simple animal models for amyotrophic lateral sclerosis drug discovery.Role of FET proteins in neurodegenerative disorders.PINK1 and Parkin are genetic modifiers for FUS-induced neurodegeneration.Reduced Insulin/IGF-1 Signaling Restores the Dynamic Properties of Key Stress Granule Proteins during Aging.More stressed out with age? Check your RNA granule aggregation.The Role of Post-Translational Modifications on Prion-Like Aggregation and Liquid-Phase Separation of FUS.Amyotrophic Lateral Sclerosis associated FUS mutation shortens mitochondria and induces neurotoxicity
P2860
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P2860
ALS mutations in FUS cause neuronal dysfunction and death in Caenorhabditis elegans by a dominant gain-of-function mechanism.
description
2011 nî lūn-bûn
@nan
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
2011年论文
@zh
2011年论文
@zh-cn
name
ALS mutations in FUS cause neu ...... nt gain-of-function mechanism.
@ast
ALS mutations in FUS cause neu ...... nt gain-of-function mechanism.
@en
type
label
ALS mutations in FUS cause neu ...... nt gain-of-function mechanism.
@ast
ALS mutations in FUS cause neu ...... nt gain-of-function mechanism.
@en
prefLabel
ALS mutations in FUS cause neu ...... nt gain-of-function mechanism.
@ast
ALS mutations in FUS cause neu ...... nt gain-of-function mechanism.
@en
P2093
P2860
P50
P356
P1476
ALS mutations in FUS cause neu ...... nt gain-of-function mechanism.
@en
P2093
Aileen Moloney
Asuka Mukai
Christopher Bohm
Clemens F Kaminski
Damian Crowther
Fiona T S Chan
Fusheng Chen
Gabriele S Kaminski Schierle
Hiroshi Suzuki
P2860
P356
10.1093/HMG/DDR417
P577
2011-09-23T00:00:00Z