Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons
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RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense interventionThere has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALShiPSC-derived iMSCs: NextGen MSCs as an advanced therapeutically active cell resource for regenerative medicineThe Use of Stem Cells to Model Amyotrophic Lateral Sclerosis and Frontotemporal Dementia: From Basic Research to Regenerative MedicineFrontotemporal Lobar Degeneration and MicroRNAsInduced Pluripotent Stem Cells as a Novel Tool in Psychiatric ResearchEpigenetic Research of Neurodegenerative Disorders Using Patient iPSC-Based ModelsAn Overview of Direct Somatic Reprogramming: The Ins and Outs of iPSCsModeling ALS and FTD with iPSC-derived neuronsRAN translation and frameshifting as translational challenges at simple repeats of human neurodegenerative disordersInduced pluripotent stem cells from ALS patients for disease modelingModeling diseases of noncoding unstable repeat expansions using mutant pluripotent stem cells.Emerging mechanisms of molecular pathology in ALSUtilizing induced pluripotent stem cells (iPSCs) to understand the actions of estrogens in human neuronsRNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosisG-quadruplexes: Emerging roles in neurodegenerative diseases and the non-coding transcriptomeApproaches to Validate and Manipulate RNA Targets with Small Molecules in CellsLoss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell deathGene expression profiling for human iPS-derived motor neurons from sporadic ALS patients reveals a strong association between mitochondrial functions and neurodegeneration.C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.C9orf72 nucleotide repeat structures initiate molecular cascades of disease.Hippocampal sclerosis dementia with the C9ORF72 hexanucleotide repeat expansionMouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal DementiaTargeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansionCharacterization of DNA hypermethylation in the cerebellum of c9FTD/ALS patientsC9orf72 hypermethylation protects against repeat expansion-associated pathology in ALS/FTD.C9orf72 amyotrophic lateral sclerosis and frontotemporal dementia: gain or loss of function?RNA-protein interactions in unstable microsatellite diseases.The emerging roles of microRNAs in the pathogenesis of frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) spectrum disordersInvestigating human disease using stem cell models.GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport.Repeat associated non-ATG (RAN) translation: new starts in microsatellite expansion disorders.Alterations in microRNA-124 and AMPA receptors contribute to social behavioral deficits in frontotemporal dementia.Stem cells on the brain: modeling neurodevelopmental and neurodegenerative diseases using human induced pluripotent stem cells.Aberrant RNA homeostasis in amyotrophic lateral sclerosis: potential for new therapeutic targets?Downregulation of microRNA-9 in iPSC-derived neurons of FTD/ALS patients with TDP-43 mutations.Gene expression signatures in motor neurone disease fibroblasts reveal dysregulation of metabolism, hypoxia-response and RNA processing functionsHuman iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viabilityDrosha inclusions are new components of dipeptide-repeat protein aggregates in FTLD-TDP and ALS C9orf72 expansion casesBromodomain inhibitors regulate the C9ORF72 locus in ALS
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P2860
Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons
description
2013 nî lūn-bûn
@nan
2013 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2013 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2013年の論文
@ja
2013年論文
@yue
2013年論文
@zh-hant
2013年論文
@zh-hk
2013年論文
@zh-mo
2013年論文
@zh-tw
2013年论文
@wuu
name
Modeling key pathological feat ...... in iPSC-derived human neurons
@ast
Modeling key pathological feat ...... in iPSC-derived human neurons
@en
type
label
Modeling key pathological feat ...... in iPSC-derived human neurons
@ast
Modeling key pathological feat ...... in iPSC-derived human neurons
@en
prefLabel
Modeling key pathological feat ...... in iPSC-derived human neurons
@ast
Modeling key pathological feat ...... in iPSC-derived human neurons
@en
P2093
P2860
P4510
P50
P1476
Modeling key pathological feat ...... in iPSC-derived human neurons
@en
P2093
Andrew R Tapper
Anna Karydas
Chantal Sellier
Eduardo Gascon
Fen-Biao Gao
Hsin Jung Chou
Hélène Tran
Nicolas Charlet-Berguerand
Sandra Almeida
Steven Degroot
P2860
P2888
P304
P356
10.1007/S00401-013-1149-Y
P577
2013-07-09T00:00:00Z
P5875
P6179
1009162788