C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD - Wikidata - awgldk - TriplyDB

C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

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From animal models to human disease: a genetic approach for personalized medicine in ALS There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALS Pathogenesis of FUS-associated ALS and FTD: insights from rodent models Glycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegeneration Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell death The ALS/FTLD associated protein C9orf72 associates with SMCR8 and WDR41 to regulate the autophagy-lysosome pathway.Loss of C9orf72 Enhances Autophagic Activity via Deregulated mTOR and TFEB Signaling C9orf72 binds SMCR8, localizes to lysosomes, and regulates mTORC1 signaling.Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS/FTLD.C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes A C9ORF72 BAC mouse model recapitulates key epigenetic perturbations of ALS/FTD Mouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal Dementia Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs Neuromuscular Junction Impairment in Amyotrophic Lateral Sclerosis: Reassessing the Role of Acetylcholinesterase.The Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded C9ORF72 Inhibits the Proteasome Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype.Poly(GP) proteins are a useful pharmacodynamic marker for C9ORF72-associated amyotrophic lateral sclerosis.New Insights on the Mechanisms of Disease Course Variability in ALS from Mutant SOD1 Mouse Models.Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.Modeling the C9ORF72 repeat expansion mutation using human induced pluripotent stem cells.Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits.C9ORF72 Regulates Stress Granule Formation and Its Deficiency Impairs Stress Granule Assembly, Hypersensitizing Cells to Stress.Genetics of FTLD: overview and what else we can expect from genetic studies.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.Nuclear transport dysfunction: a common theme in amyotrophic lateral sclerosis and frontotemporal dementia.Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies.Precision medicine, genomics and drug discovery.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.Decoding ALS: from genes to mechanism.High content analysis in amyotrophic lateral sclerosis.ALS/FTLD: experimental models and reality.The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.New developments in RAN translation: insights from multiple diseases.Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size.Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia.C9ORF72 is a GDP/GTP exchange factor for Rab8 and Rab39 and regulates autophagy.In-depth clinico-pathological examination of RNA foci in a large cohort of C9ORF72 expansion carriers.Spinal poly-GA inclusions in a C9orf72 mouse model trigger motor deficits and inflammation without neuron loss.

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

http://www.wikidata.org/entity/Q36353773

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2015年の論文

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2015年学术文章

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2015年学术文章

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2015年学术文章

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2015年学术文章

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2015年学术文章

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2015年學術文章

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2015年學術文章

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2015年學術文章

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

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J.NEURON.2015.10.027

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C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD

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A K M G Muhammad

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Andrew Austin

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Cathleen M Lutz

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Deepti Lall

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Dennis W Dickson

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Edward B Lee

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Elaine Y Liu

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Jacqueline G O'Rourke

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Janet Cady

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Jonah Zarrow

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P2860

C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

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

Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS

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

Mouse models of frontotemporal dementia

C9orf72 nucleotide repeat structures initiate molecular cascades of disease.

Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion

TAR DNA-binding protein 43 in neurodegenerative disease.

Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress.

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scholarly article

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10.1016/J.NEURON.2015.10.027

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5

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88

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Laurent Bogdanik

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2015-12-01T00:00:00Z

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26637796

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amyotrophic lateral sclerosis

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4672384

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