Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS. - Test2 - elhamkhani - TriplyDB

Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS.

Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS.

http://www.wikidata.org/entity/Q34467825

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Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration Structural basis of nucleic-acid recognition and double-strand unwinding by the essential neuronal protein Pur-alpha TDP-43 and FUS en route from the nucleus to the cytoplasm.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 Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation Identifying proteins that bind to specific RNAs - focus on simple repeat expansion diseases.Marked Differences in C9orf72 Methylation Status and Isoform Expression between C9/ALS Human Embryonic and Induced Pluripotent Stem Cells.Structural Characteristics of Simple RNA Repeats Associated with Disease and their Deleterious Protein Interactions.Old versus New Mechanisms in the Pathogenesis of ALS.The small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.ALS: Recent Developments from Genetics Studies.Nuclear trafficking in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.A short conserved motif in ALYREF directs cap- and EJC-dependent assembly of export complexes on spliced mRNAs The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.Control of mRNA Translation in ALS Proteinopathy Energy Homeostasis and Abnormal RNA Metabolism in Amyotrophic Lateral Sclerosis The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains.RNA Misprocessing in C9orf72-Linked Neurodegeneration.In-depth clinico-pathological examination of RNA foci in a large cohort of C9ORF72 expansion carriers.Failure to Deliver and Translate-New Insights into RNA Dysregulation in ALS.PURA, the gene encoding Pur-alpha, member of an ancient nucleic acid-binding protein family with mammalian neurological functions.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.The roles of intrinsic disorder-based liquid-liquid phase transitions in the "Dr. Jekyll-Mr. Hyde" behavior of proteins involved in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis.Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.Unraveling the Role of RNA Mediated Toxicity of C9orf72 Repeats in C9-FTD/ALS.mRNP assembly, axonal transport, and local translation in neurodegenerative diseases.Assay to Measure Nucleocytoplasmic Transport in Real Time within Motor Neuron-like NSC-34 Cells.Perturbed autophagy and DNA repair converge to promote neurodegeneration in amyotrophic lateral sclerosis and dementia.Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA.

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Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS.

http://www.wikidata.org/entity/Q34467825

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2015 nî lūn-bûn

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2015 թուականի Մարտին հրատարակուած գիտական յօդուած

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2015 թվականի մարտին հրատարակված գիտական հոդված

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Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.

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Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.

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Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.

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Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.

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Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.

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Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.

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Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.

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Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.

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Nuclear accumulation of mRNAs ...... cellular model of C9orf72 ALS.

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Alessandra Giorgi

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Alessia Serrano

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Claudia Bagni

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Francesca Bozzo

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Gianluca Cestra

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Maria Eugenia Schininà

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Monica Nencini

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Simona Rossi

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Tilmann Achsel

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Valeria Gerbino

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10.1242/JCS.165332

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9

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128

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Laura Di Francesco

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Maria Teresa Carri

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2015-03-18T00:00:00Z

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25788698

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amyotrophic lateral sclerosis