Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability - Wikidata - awgldk - TriplyDB

Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability

Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability

http://www.wikidata.org/entity/Q35114316

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The Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative Medicine Emerging Roles of Filopodia and Dendritic Spines in Motoneuron Plasticity during Development and Disease Epigenetic Research of Neurodegenerative Disorders Using Patient iPSC-Based Models ALS Patient Stem Cells for Unveiling Disease Signatures of Motoneuron Susceptibility: Perspectives on the Deadly Mitochondria, ER Stress and Calcium Triad Modeling ALS and FTD with iPSC-derived neurons INaP selective inhibition reverts precocious inter- and motorneurons hyperexcitability in the Sod1-G93R zebrafish ALS model Modeling ALS with motor neurons derived from human induced pluripotent stem cells Axonal Dysfunction Precedes Motor Neuronal Death in Amyotrophic Lateral Sclerosis The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.Gene expression profiling for human iPS-derived motor neurons from sporadic ALS patients reveals a strong association between mitochondrial functions and neurodegeneration.Disrupted neuronal maturation in Angelman syndrome-derived induced pluripotent stem cells.Genetic Correction of SOD1 Mutant iPSCs Reveals ERK and JNK Activated AP1 as a Driver of Neurodegeneration in Amyotrophic Lateral Sclerosis.Induced Pluripotent Stem Cells: Global Research Trends.Probing disorders of the nervous system using reprogramming approaches.Episomal Induced Pluripotent Stem Cells Promote Functional Recovery of Transected Murine Peripheral Nerve.Using human induced pluripotent stem cells to model cerebellar disease: hope and hype.Maturation and electrophysiological properties of human pluripotent stem cell-derived oligodendrocytes Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs Marked changes in dendritic structure and spine density precede significant neuronal death in vulnerable cortical pyramidal neuron populations in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis Marked Differences in C9orf72 Methylation Status and Isoform Expression between C9/ALS Human Embryonic and Induced Pluripotent Stem Cells.Induced pluripotent stem cells from patients with focal cortical dysplasia and refractory epilepsy.The Role of TDP-43 in Alzheimer's Disease.Inhibitory dysfunction in amyotrophic lateral sclerosis: future therapeutic opportunities.Modeling the C9ORF72 repeat expansion mutation using human induced pluripotent stem cells.Impairments in Motor Neurons, Interneurons and Astrocytes Contribute to Hyperexcitability in ALS: Underlying Mechanisms and Paths to Therapy.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.Modeling simple repeat expansion diseases with iPSC technology.Review: Induced pluripotent stem cell models of frontotemporal dementia.Axonal Excitability in Amyotrophic Lateral Sclerosis : Axonal Excitability in ALS.Induced pluripotent stem cell technology: a decade of progress.Synapse Dysfunction of Layer V Pyramidal Neurons Precedes Neurodegeneration in a Mouse Model of TDP-43 Proteinopathies.Autoantibody pathogenicity in a multifocal motor neuropathy induced pluripotent stem cell-derived model.4-Aminopyridine Induced Activity Rescues Hypoexcitable Motor Neurons from Amyotrophic Lateral Sclerosis Patient-Derived Induced Pluripotent Stem Cells.Simple Derivation of Spinal Motor Neurons from ESCs/iPSCs Using Sendai Virus Vectors.ALS disrupts spinal motor neuron maturation and aging pathways within gene co-expression networks The modulation of two motor behaviors by persistent sodium currents in Xenopus laevis tadpoles.Aberrant association of misfolded SOD1 with Na(+)/K(+)ATPase-α3 impairs its activity and contributes to motor neuron vulnerability in ALS.Cortical hyperexcitability in patients with C9ORF72 mutations: Relationship to phenotype.A Stem Cell Model of the Motor Circuit Uncouples Motor Neuron Death from Hyperexcitability Induced by SMN Deficiency.Do We Have a Channel Solution for ALS?

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Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability

http://www.wikidata.org/entity/Q35114316

description

2015 nî lūn-bûn

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2015 թուականի Յունուարին հրատարակուած գիտական յօդուած

@hyw

2015 թվականի հունվարին հրատարակված գիտական հոդված

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2015年の論文

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2015年論文

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2015年論文

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2015年論文

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2015年論文

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2015年論文

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2015年论文

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name

Human iPSC-derived motoneurons ...... despite maintaining viability

@ast

Human iPSC-derived motoneurons ...... despite maintaining viability

@en

type

label

Human iPSC-derived motoneurons ...... despite maintaining viability

@ast

Human iPSC-derived motoneurons ...... despite maintaining viability

@en

prefLabel

Human iPSC-derived motoneurons ...... despite maintaining viability

@ast

Human iPSC-derived motoneurons ...... despite maintaining viability

@en

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Nature Communications

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Human iPSC-derived motoneurons ...... despite maintaining viability

@en

P2093

Elaine M Cleary

http://www.w3.org/2001/XMLSchema#string

Imbisaat Geti

http://www.w3.org/2001/XMLSchema#string

Karen Burr

http://www.w3.org/2001/XMLSchema#string

Shyamanga Borooah

http://www.w3.org/2001/XMLSchema#string

P2860

RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

Targeted gene correction of α1-antitrypsin deficiency in induced pluripotent stem cells

State of play in amyotrophic lateral sclerosis genetics

Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1.

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

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

Induced pluripotent stem cells generated from patients with ALS can be differentiated into motor neurons

ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.

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5999

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10.1038/NCOMMS6999

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P478

6

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Gareth B. Miles

Ludovic Vallier

Siddharthan Chandran

Christopher E Shaw

Anna-Claire Devlin

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2015-01-12T00:00:00Z

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270910554

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1030797096

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25580746

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amyotrophic lateral sclerosis

P932

4338554

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