Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
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TARDBP mutation analysis in TDP-43 proteinopathies and deciphering the toxicity of mutant TDP-43From animal models to human disease: a genetic approach for personalized medicine in ALSMolecular motor proteins and amyotrophic lateral sclerosisPathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersThe role of the immune system in neurodegenerative disorders: Adaptive or maladaptive?Mitochondrial dysfunction in amyotrophic lateral sclerosis - a valid pharmacological target?Does a loss of TDP-43 function cause neurodegeneration?Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutationsALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) mice.Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.TDP-43 suppresses tau expression via promoting its mRNA instability.Abnormalities of Mitochondrial Dynamics in Neurodegenerative Diseases.Drosophila 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 influence of pathological mutations and proline substitutions in TDP-43 glycine-rich peptides on its amyloid properties and cellular toxicity.The pathological phenotypes of human TDP-43 transgenic mouse models are independent of downregulation of mouse Tdp-43Opposing roles of p38 and JNK in a Drosophila model of TDP-43 proteinopathy reveal oxidative stress and innate immunity as pathogenic components of neurodegeneration.Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viabilityAllele-specific knockdown of ALS-associated mutant TDP-43 in neural stem cells derived from induced pluripotent stem cellsMitochondrial dynamic abnormalities in amyotrophic lateral sclerosis.Rodent Models of Amyotrophic Lateral SclerosisLoss of RAD-23 Protects Against Models of Motor Neuron Disease by Enhancing Mutant Protein ClearanceAsymmetric pathology in primary progressive aphasia with progranulin mutations and TDP inclusions.Rodent models of TDP-43: recent advances.Two mutations G335D and Q343R within the amyloidogenic core region of TDP-43 influence its aggregation and inclusion formation.The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity.RNA-mediated toxicity in neurodegenerative diseaseThe ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons.Disease animal models of TDP-43 proteinopathy and their pre-clinical applications.Transgenic mice overexpressing the ALS-linked protein Matrin 3 develop a profound muscle phenotype.Divergent phenotypes in mutant TDP-43 transgenic mice highlight potential confounds in TDP-43 transgenic modeling.Cytoplasmic Relocalization of TAR DNA-Binding Protein 43 Is Not Sufficient to Reproduce Cellular Pathologies Associated with ALS In vitro.Mitochondrion-mediated cell death: dissecting yeast apoptosis for a better understanding of neurodegeneration.Protein aggregation in amyotrophic lateral sclerosis.AMPK-mediated regulation of neuronal metabolism and function in brain diseases.Frontotemporal Lobar Degeneration: Mechanisms and Therapeutic Strategies.TDP-43 Depletion in Microglia Promotes Amyloid Clearance but Also Induces Synapse Loss.Advances in the Development of Disease-Modifying Treatments for Amyotrophic Lateral Sclerosis.Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts.
P2860
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P2860
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
description
2011 nî lūn-bûn
@nan
2011 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
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
name
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
@ast
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
@en
type
label
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
@ast
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
@en
prefLabel
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
@ast
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
@en
P2093
P2860
P356
P1476
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
@en
P2093
Caroline Stetler
Jada Lewis
Wen-Lang Lin
Xiangkun Cao
Yong-Jie Zhang
P2860
P2888
P356
10.1186/1750-1326-6-73
P577
2011-10-26T00:00:00Z
P5875
P6179
1018624284