Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS
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There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALSRAN translation and frameshifting as translational challenges at simple repeats of human neurodegenerative disordersTherapeutic and diagnostic challenges for frontotemporal dementiaModeling diseases of noncoding unstable repeat expansions using mutant pluripotent stem cells.Frontotemporal dementia: a bridge between dementia and neuromuscular diseaseEmerging mechanisms of molecular pathology in ALSRNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosisG-quadruplexes: Emerging roles in neurodegenerative diseases and the non-coding transcriptomeDrosophila as an In Vivo Model for Human Neurodegenerative DiseaseGlycine-alanine dipeptide repeat protein contributes to toxicity in a zebrafish model of C9orf72 associated neurodegenerationApproaches 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 deathReduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein depositionIntrinsic disorder in proteins involved in amyotrophic lateral sclerosis.Neuropathology of Amyotrophic Lateral Sclerosis and Its Variants.C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Structure and Dynamics of DNA and RNA Double Helices Obtained from the GGGGCC and CCCCGG Hexanucleotide Repeats That Are the Hallmark of C9FTD/ALS Diseases.RNA metabolism in neurodegenerative disease.Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansionsAbnormal expression of homeobox genes and transthyretin in C9ORF72 expansion carriersPoly-GP in cerebrospinal fluid links C9orf72-associated dipeptide repeat expression to the asymptomatic phase of ALS/FTD.Mouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal DementiaCell-type specific differences in promoter activity of the ALS-linked C9orf72 mouse ortholog.Characterization of DNA hypermethylation in the cerebellum of c9FTD/ALS patientsThe role of long non-coding RNAs in neurodevelopment, brain function and neurological diseaseAggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress.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 disordersGGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport.Discovery of a biomarker and lead small molecules to target r(GGGGCC)-associated defects in c9FTD/ALS.Repeat associated non-ATG (RAN) translation: new starts in microsatellite expansion disorders.A network of RNA and protein interactions in Fronto Temporal Dementia.Frontotemporal lobar degeneration: defining phenotypic diversity through personalized medicine.Repeat-associated non-AUG translation and its impact in neurodegenerative disease.The Spectrum of C9orf72-mediated Neurodegeneration and Amyotrophic Lateral Sclerosis.Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy.Dipeptide repeat protein inclusions are rare in the spinal cord and almost absent from motor neurons in C9ORF72 mutant amyotrophic lateral sclerosis and are unlikely to cause their degeneration.RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndromeRNA Structures as Mediators of Neurological Diseases and as Drug Targets.
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Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS
description
article científic
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article scientifique
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articolo scientifico
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artigo científico
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bilimsel makale
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scientific article published on 16 October 2013
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Antisense transcripts of the e ...... n-ATG translation in c9FTD/ALS
@en
Antisense transcripts of the e ...... -ATG translation in c9FTD/ALS.
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type
label
Antisense transcripts of the e ...... n-ATG translation in c9FTD/ALS
@en
Antisense transcripts of the e ...... -ATG translation in c9FTD/ALS.
@nl
prefLabel
Antisense transcripts of the e ...... n-ATG translation in c9FTD/ALS
@en
Antisense transcripts of the e ...... -ATG translation in c9FTD/ALS.
@nl
P2093
P2860
P50
P1476
Antisense transcripts of the e ...... n-ATG translation in c9FTD/ALS
@en
P2093
Danielle M Cosio
Jeannie Chew
Judith H Dunmore
Karen Jansen-West
Kevin B Boylan
Lillian Daughrity
Monica Castanedes-Casey
Peter E A Ash
Tania F Gendron
Thomas Caulfield
P2860
P2888
P304
P356
10.1007/S00401-013-1192-8
P577
2013-10-16T00:00:00Z