The disease-associated r(GGGGCC)n repeat from the C9orf72 gene forms tract length-dependent uni- and multimolecular RNA G-quadruplex structures
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RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense interventionC9orf72 expansions are the most common genetic cause of Huntington disease phenocopiesThere has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALSDysregulated axonal RNA translation in amyotrophic lateral sclerosisRAN translation and frameshifting as translational challenges at simple repeats of human neurodegenerative disordersPathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersModeling diseases of noncoding unstable repeat expansions using mutant pluripotent stem cells.RNA-mediated pathogenic mechanisms in polyglutamine diseases and amyotrophic lateral sclerosisG-quadruplexes: Emerging roles in neurodegenerative diseases and the non-coding transcriptomeC9orf72-related disorders: expanding the clinical and genetic spectrum of neurodegenerative diseasesRNA phase transitions in repeat expansion disorders.DNA Replication Dynamics of the GGGGCC Repeat of the C9orf72 Gene.DNA secondary structure at chromosomal fragile sites in human disease.Processing of double-R-loops in (CAG)·(CTG) and C9orf72 (GGGGCC)·(GGCCCC) repeats causes instability.Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neuronsTopology of a G-quadruplex DNA formed by C9orf72 hexanucleotide repeats associated with ALS and FTD.C9orf72 nucleotide repeat structures initiate molecular cascades of disease.Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansionsMouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal DementiaMolecular network analysis suggests a logical hypothesis for the pathological role of c9orf72 in amyotrophic lateral sclerosis/frontotemporal dementia.Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress.C9orf72 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.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.tRNA-derived G-quadruplex protects motor neurons.Aberrant RNA homeostasis in amyotrophic lateral sclerosis: potential for new therapeutic targets?Drosha inclusions are new components of dipeptide-repeat protein aggregates in FTLD-TDP and ALS C9orf72 expansion casesA network of RNA and protein interactions in Fronto Temporal Dementia.G-quadruplex structures formed by expanded hexanucleotide repeat RNA and DNA from the neurodegenerative disease-linked C9orf72 gene efficiently sequester and activate heme.The mouse C9ORF72 ortholog is enriched in neurons known to degenerate in ALS and FTD.The Spectrum of C9orf72-mediated Neurodegeneration and Amyotrophic Lateral Sclerosis.Repeat Associated Non-AUG Translation (RAN Translation) Dependent on Sequence Downstream of the ATXN2 CAG RepeatRepeat-mediated epigenetic dysregulation of the FMR1 gene in the fragile X-related disorders.RNA-binding protein misregulation in microsatellite expansion disordersRNA Structures as Mediators of Neurological Diseases and as Drug Targets.MBNL Sequestration by Toxic RNAs and RNA Misprocessing in the Myotonic Dystrophy BrainEmerging role of RNA•DNA hybrids in C9orf72-linked neurodegeneration.Structural Analysis of G-Quadruplex Formation at the Human MEST Promoter.
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P2860
The disease-associated r(GGGGCC)n repeat from the C9orf72 gene forms tract length-dependent uni- and multimolecular RNA G-quadruplex structures
description
2013 nî lūn-bûn
@nan
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
2013年论文
@zh
2013年论文
@zh-cn
name
The disease-associated r(GGGGC ...... ar RNA G-quadruplex structures
@ast
The disease-associated r(GGGGC ...... ar RNA G-quadruplex structures
@en
type
label
The disease-associated r(GGGGC ...... ar RNA G-quadruplex structures
@ast
The disease-associated r(GGGGC ...... ar RNA G-quadruplex structures
@en
prefLabel
The disease-associated r(GGGGC ...... ar RNA G-quadruplex structures
@ast
The disease-associated r(GGGGC ...... ar RNA G-quadruplex structures
@en
P2093
P2860
P356
P1476
The disease-associated r(GGGGC ...... ar RNA G-quadruplex structures
@en
P2093
Bita Zamiri
Christopher E Pearson
Kaalak Reddy
Robert B Macgregor
Sabrina Y R Stanley
P2860
P304
P356
10.1074/JBC.C113.452532
P407
P577
2013-02-19T00:00:00Z