Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS. - Wikidata - awgldk - TriplyDB

Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS.

Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS.

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From animal models to human disease: a genetic approach for personalized medicine in ALS Inside out: the role of nucleocytoplasmic transport in ALS and FTLD There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALS The ALS/FTLD associated protein C9orf72 associates with SMCR8 and WDR41 to regulate the autophagy-lysosome pathway.Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition 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.Modelling amyotrophic lateral sclerosis: progress and possibilities Mouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal Dementia SRSF1-dependent nuclear export inhibition of C9ORF72 repeat transcripts prevents neurodegeneration and associated motor deficits.Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity.GGGGCC microsatellite RNA is neuritically localized, induces branching defects, and perturbs transport granule function Retention of hexanucleotide repeat-containing intron in C9orf72 mRNA: implications for the pathogenesis of ALS/FTD Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs Nucleocytoplasmic transport in C9orf72-mediated ALS/FTD.The Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded C9ORF72 Inhibits the Proteasome Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106.Drosophila melanogaster As a Model Organism to Study RNA Toxicity of Repeat Expansion-Associated Neurodegenerative and Neuromuscular Diseases Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD.Evidence that C9ORF72 Dipeptide Repeat Proteins Associate with U2 snRNP to Cause Mis-splicing in ALS/FTD Patients.Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.RAN translation-What makes it run?Nuclear transport dysfunction: a common theme in amyotrophic lateral sclerosis and frontotemporal dementia.Simple animal models for amyotrophic lateral sclerosis drug discovery.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.Molecular Genetics of Neurodegenerative Dementias.Modelling C9orf72 dipeptide repeat proteins of a physiologically relevant size.RNA Misprocessing in C9orf72-Linked Neurodegeneration.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.Antibodies inhibit transmission and aggregation of C9orf72 poly-GA dipeptide repeat proteins Biological Spectrum of Amyotrophic Lateral Sclerosis Prions.Rethinking Unconventional Translation in Neurodegeneration.Sense-encoded poly-GR dipeptide repeat proteins correlate to neurodegeneration and uniquely co-localize with TDP-43 in dendrites of repeat-expanded C9orf72 amyotrophic lateral sclerosis.RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response.C9orf72 poly GA RAN-translated protein plays a key role in amyotrophic lateral sclerosis via aggregation and toxicity.C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity.C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2α phosphorylation.

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Differential Toxicity of Nuclear RNA Foci versus Dipeptide Repeat Proteins in a Drosophila Model of C9ORF72 FTD/ALS.

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Fen-Biao Gao

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Helene Tran

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Jeffrey A Nickerson

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Jill Moore

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Sandra Almeida

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Tania F Gendron

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UmaDevi Chalasani

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Xing Du

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Yubing Lu

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Zhiping Weng

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P2860

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

Site-specific transformation of Drosophila via phiC31 integrase-mediated cassette exchange

Pyrosequencing sheds light on DNA sequencing

Age-dependent deterioration of nuclear pore complexes causes a loss of nuclear integrity in postmitotic cells

Methods to assay Drosophila behavior.

Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons

C9orf72 nucleotide repeat structures initiate molecular cascades of disease.

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1207-1214

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Leonard Petrucelli

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amyotrophic lateral sclerosis

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