FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration
about
Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradationFrom animal models to human disease: a genetic approach for personalized medicine in ALSMechanisms of FUS mutations in familial amyotrophic lateral sclerosisDysregulated axonal RNA translation in amyotrophic lateral sclerosisConverging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisThe function of RNA-binding proteins at the synapse: implications for neurodegenerationMitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?Early lethality and neuronal proteinopathy in mice expressing cytoplasm-targeted FUS that lacks the RNA recognition motif.Structural 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 modelsThe neurogenetics of alternative splicingEmerging mechanisms of molecular pathology in ALSOxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutationsFUS Interacts with HSP60 to Promote Mitochondrial DamageAberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosisReduced 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 cytoplasmExpression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in ratsCharacterization of genetic loss-of-function of Fus in zebrafish.CRISPR/Cas9-mediated targeted gene correction in amyotrophic lateral sclerosis patient iPSCs.AAV2 mediated retrograde transduction of corticospinal motor neurons reveals initial and selective apical dendrite degeneration in ALS.Mutant human FUS Is ubiquitously mislocalized and generates persistent stress granules in primary cultured transgenic zebrafish cellsRNA metabolism in neurodegenerative disease.The 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 homeostasisTDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic miceALS-associated FUS mutations result in compromised FUS alternative splicing and autoregulation.A 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.Understanding the role of TDP-43 and FUS/TLS in ALS and beyond.Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1.Identification of pharmacological targets in amyotrophic lateral sclerosis through genomic analysis of deregulated genes and pathways.Severe respiratory changes at end stage in a FUS-induced disease state in adult rats.Mechanisms of disease in frontotemporal lobar degeneration: gain of function versus loss of function effectsEntorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats.XBP1 depletion precedes ubiquitin aggregation and Golgi fragmentation in TDP-43 transgenic rats.ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.
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P2860
FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration
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2011 nî lūn-bûn
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2011 թուականի Մարտին հրատարակուած գիտական յօդուած
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2011 թվականի մարտին հրատարակված գիտական հոդված
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2011年の論文
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2011年論文
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2011年論文
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2011年論文
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2011年論文
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2011年論文
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2011年论文
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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P2860
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FUS transgenic rats develop th ...... ntotemporal lobar degeneration
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Hongxia Zhou
Jianbin Tong
Xiaotao Wei
Xu-Gang Xia
Yong-Jian Liu
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P356
10.1371/JOURNAL.PGEN.1002011
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2011-03-01T00:00:00Z