Motor neuron apoptosis and neuromuscular junction perturbation are prominent features in a Drosophila model of Fus-mediated ALS
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A loss of FUS/TLS function leads to impaired cellular proliferationDrosophila as an In Vivo Model for Human Neurodegenerative DiseaseFUS-NLS/Transportin 1 complex structure provides insights into the nuclear targeting mechanism of FUS and the implications in ALSThe 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.Mechanisms, models and biomarkers in amyotrophic lateral sclerosisA fruitful endeavor: modeling ALS in the fruit flyModel systems of motor neuron diseases as a platform for studying pathogenic mechanisms and searching for therapeutic agents.Subcellular localization and RNAs determine FUS architecture in different cellular compartments.Severe respiratory changes at end stage in a FUS-induced disease state in adult rats.Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis.The RRM domain of human fused in sarcoma protein reveals a non-canonical nucleic acid binding site.Genome wide array analysis indicates that an amyotrophic lateral sclerosis mutation of FUS causes an early increase of CAMK2N2 in vitro.Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice.Defects in synapse structure and function precede motor neuron degeneration in Drosophila models of FUS-related ALS.FUsed in sarcoma is a novel regulator of manganese superoxide dismutase gene transcription.Protein aggregation in amyotrophic lateral sclerosis.The role of FUS gene variants in neurodegenerative diseases.Ataxin-2 interacts with FUS and intermediate-length polyglutamine expansions enhance FUS-related pathology in amyotrophic lateral sclerosis.FET proteins regulate lifespan and neuronal integrity.Coordinated movement, neuromuscular synaptogenesis and trans-synaptic signaling defects in Drosophila galactosemia models.Pur-alpha functionally interacts with FUS carrying ALS-associated mutations.Overelaborated synaptic architecture and reduced synaptomatrix glycosylation in a Drosophila classic galactosemia disease model.Neuromuscular Junction Dismantling in Amyotrophic Lateral Sclerosis.FUS toxicity is rescued by the modulation of lncRNA hsrω expression in Drosophila melanogaster.Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice.Muscle atrophy is associated with cervical spinal motoneuron loss in BACHD mouse model for Huntington's disease.A Drosophila model of ALS reveals a partial loss of function of causative human PFN1 mutants.Yeast as a Model to Unravel Mechanisms Behind FUS Toxicity in Amyotrophic Lateral Sclerosis.
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P2860
Motor neuron apoptosis and neuromuscular junction perturbation are prominent features in a Drosophila model of Fus-mediated ALS
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
Motor neuron apoptosis and neu ...... hila model of Fus-mediated ALS
@ast
Motor neuron apoptosis and neu ...... hila model of Fus-mediated ALS
@en
type
label
Motor neuron apoptosis and neu ...... hila model of Fus-mediated ALS
@ast
Motor neuron apoptosis and neu ...... hila model of Fus-mediated ALS
@en
prefLabel
Motor neuron apoptosis and neu ...... hila model of Fus-mediated ALS
@ast
Motor neuron apoptosis and neu ...... hila model of Fus-mediated ALS
@en
P2093
P2860
P356
P1476
Motor neuron apoptosis and neu ...... hila model of Fus-mediated ALS
@en
P2093
Haining Zhu
Liuqing Yang
Ruohan Xia
Yajuan Liu
P2860
P2888
P356
10.1186/1750-1326-7-10
P577
2012-03-24T00:00:00Z
P5875
P6179
1036705691