TDP-43-mediated neuron loss in vivo requires RNA-binding activity
about
Altered ribostasis: RNA-protein granules in degenerative disordersConjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUSFrom animal models to human disease: a genetic approach for personalized medicine in ALSPathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersPotential mechanisms of progranulin-deficient FTLDTDP-43 Proteinopathy and ALS: Insights into Disease Mechanisms and Therapeutic Targets.Drosophila as an In Vivo Model for Human Neurodegenerative DiseaseIdentification of genetic modifiers of TDP-43 neurotoxicity in DrosophilaTDP-43 is a transcriptional repressor: the testis-specific mouse acrv1 gene is a TDP-43 target in vivo.Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in ratsRNA metabolism in neurodegenerative disease.3'UTRs take a long shot in the brain.TDP-43 regulates Drosophila neuromuscular junctions growth by modulating Futsch/MAP1B levels and synaptic microtubules organizationDrosophila lines with mutant and wild type human TDP-43 replacing the endogenous gene reveals phosphorylation and ubiquitination in mutant lines in the absence of viability or lifespan defects.The mitochondrial chaperone protein TRAP1 mitigates α-Synuclein toxicityThe aggregation and neurotoxicity of TDP-43 and its ALS-associated 25 kDa fragment are differentially affected by molecular chaperones in Drosophila.The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease.Motor neurons and glia exhibit specific individualized responses to TDP-43 expression in a Drosophila model of amyotrophic lateral sclerosis.Inhibition of TDP-43 aggregation by nucleic acid bindingRNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43.Opposing roles of p38 and JNK in a Drosophila model of TDP-43 proteinopathy reveal oxidative stress and innate immunity as pathogenic components of neurodegeneration.A yeast model for polyalanine-expansion aggregation and toxicityLoss of nuclear TDP-43 in amyotrophic lateral sclerosis (ALS) causes altered expression of splicing machinery and widespread dysregulation of RNA splicing in motor neurones.UBE2E ubiquitin-conjugating enzymes and ubiquitin isopeptidase Y regulate TDP-43 protein ubiquitination.U1 snRNP is mislocalized in ALS patient fibroblasts bearing NLS mutations in FUS and is required for motor neuron outgrowth in zebrafishPPAR gamma activation is neuroprotective in a Drosophila model of ALS based on TDP-43.A fruitful endeavor: modeling ALS in the fruit flyLinking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis.Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration.PABPN1 suppresses TDP-43 toxicity in ALS disease models.Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease modelsThe RRM domain of human fused in sarcoma protein reveals a non-canonical nucleic acid binding site.RNA-binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutationsStress granules as crucibles of ALS pathogenesis.Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish.Misregulated RNA processing in amyotrophic lateral sclerosis.RNA-binding proteins in neurodegenerative disease: TDP-43 and beyond.Deciphering amyotrophic lateral sclerosis: what phenotype, neuropathology and genetics are telling us about pathogenesis.
P2860
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P2860
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
description
2010 nî lūn-bûn
@nan
2010 թուականին հրատարակուած գիտական յօդուած
@hyw
2010 թվականին 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
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@ast
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@en
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@nl
type
label
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@ast
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@en
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@nl
prefLabel
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@ast
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@en
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@nl
P2093
P2860
P3181
P1433
P1476
TDP-43-mediated neuron loss in vivo requires RNA-binding activity
@en
P2093
Aaron Voigt
Daniel Müller
David Herholz
Kavita Kaur
Peter Karsten
Philipp J Kahle
Stephanie S Weber
Till Marquardt
P2860
P304
P3181
P356
10.1371/JOURNAL.PONE.0012247
P407
P577
2010-01-01T00:00:00Z