FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration - Test2 - elhamkhani - TriplyDB

FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration

FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration

http://www.wikidata.org/entity/Q27342067

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Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradation From animal models to human disease: a genetic approach for personalized medicine in ALS Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis Dysregulated axonal RNA translation in amyotrophic lateral sclerosis Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis The function of RNA-binding proteins at the synapse: implications for neurodegeneration Mitochondrial 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 models The neurogenetics of alternative splicing Emerging mechanisms of molecular pathology in ALS Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations FUS Interacts with HSP60 to Promote Mitochondrial Damage Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis Reduced 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 cytoplasm Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats Characterization 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 cells RNA 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 homeostasis TDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic mice ALS-associated FUS mutations result in compromised FUS alternative splicing and autoregulation.A fruitful endeavor: modeling ALS in the fruit fly Model 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 effects Entorhinal 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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FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration

http://www.wikidata.org/entity/Q27342067

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2011 nî lūn-bûn

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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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Cao Huang

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Dian Wang

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Han Chen

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Hongxia Zhou

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Jianbin Tong

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Xiaotao Wei

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Xu-Gang Xia

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Yong-Jian Liu

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P2860

Oncoprotein TLS interacts with serine-arginine proteins involved in RNA splicing

Fusion of the dominant negative transcription regulator CHOP with a novel gene FUS by translocation t(12;16) in malignant liposarcoma

Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

Induced ncRNAs allosterically modify RNA-binding proteins in cis to inhibit transcription

A new subtype of frontotemporal lobar degeneration with FUS pathology

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6

TLS (FUS) binds RNA in vivo and engages in nucleo-cytoplasmic shuttling

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amyotrophic lateral sclerosis

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