Sequestration of DROSHA and DGCR8 by expanded CGG RNA repeats alters microRNA processing in fragile X-associated tremor/ataxia syndrome. - Wikidata - awgldk - TriplyDB

Sequestration of DROSHA and DGCR8 by expanded CGG RNA repeats alters microRNA processing in fragile X-associated tremor/ataxia syndrome.

Sequestration of DROSHA and DGCR8 by expanded CGG RNA repeats alters microRNA processing in fragile X-associated tremor/ataxia syndrome.

http://www.wikidata.org/entity/Q40221428

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Use of model systems to understand the etiology of fragile X-associated primary ovarian insufficiency (FXPOI)The DGCR8 RNA-binding heme domain recognizes primary microRNAs by clamping the hairpin RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention From Genetics to Epigenetics: New Perspectives in Tourette Syndrome Research Fragile X spectrum disorders Fragile X-associated tremor/ataxia syndrome.The multiple molecular facets of fragile X-associated tremor/ataxia syndrome Repeat-mediated genetic and epigenetic changes at the FMR1 locus in the Fragile X-related disorders The promise and perils of HDAC inhibitors in neurodegeneration Overexpression of NF90-NF45 Represses Myogenic MicroRNA Biogenesis, Resulting in Development of Skeletal Muscle Atrophy and Centronuclear Muscle Fibers Transcription-associated R-loop formation across the human FMR1 CGG-repeat region Inhibition of Non-ATG Translational Events in Cells via Covalent Small Molecules Targeting RNA Premutation in the Fragile X Mental Retardation 1 (FMR1) Gene Affects Maternal Zn-milk and Perinatal Brain Bioenergetics and Scaffolding.CGG allele size somatic mosaicism and methylation in FMR1 premutation alleles.Fragile X-Associated Tremor/Ataxia Syndrome: From Molecular Pathogenesis to Development of Therapeutics.RNA metabolism in neurodegenerative disease.Reduced excitatory amino acid transporter 1 and metabotropic glutamate receptor 5 expression in the cerebellum of fragile X mental retardation gene 1 premutation carriers with fragile X-associated tremor/ataxia syndrome.Associated features in females with an FMR1 premutation Approaches to identify genetic variants that influence the risk for onset of fragile X-associated primary ovarian insufficiency (FXPOI): a preliminary study.Mouse models of the fragile X premutation and fragile X-associated tremor/ataxia syndrome Expression of an expanded CGG-repeat RNA in a single pair of primary sensory neurons impairs olfactory adaptation in Caenorhabditis elegans.Genomic studies in fragile X premutation carriers.RNA-protein interactions in unstable microsatellite diseases.The emerging roles of microRNAs in the pathogenesis of frontotemporal dementia-amyotrophic lateral sclerosis (FTD-ALS) spectrum disorders CGG repeats in RNA modulate expression of TDP-43 in mouse and fly models of fragile X tremor ataxia syndrome A superfolding Spinach2 reveals the dynamic nature of trinucleotide repeat-containing RNA Fragile X mental retardation protein expression in Alzheimer's disease Molecular Advances Leading to Treatment Implications for Fragile X Premutation Carriers Targeting the r(CGG) repeats that cause FXTAS with modularly assembled small molecules and oligonucleotides Memantine for fragile X-associated tremor/ataxia syndrome: a randomized, double-blind, placebo-controlled trial.Current research, diagnosis, and treatment of fragile X-associated tremor/ataxia syndrome.Dysregulated iron metabolism in the choroid plexus in fragile X-associated tremor/ataxia syndrome Immune dysregulation as a cause of autoinflammation in fragile X premutation carriers: link between FMRI CGG repeat number and decreased cytokine responses.Drosha inclusions are new components of dipeptide-repeat protein aggregates in FTLD-TDP and ALS C9orf72 expansion cases Live-cell imaging of mammalian RNAs with Spinach2.Repeat-associated non-AUG translation and its impact in neurodegenerative disease.Prevalence of restless legs syndrome and sleep quality in carriers of the fragile X premutation.High-Throughput Screening to Identify Compounds That Increase Fragile X Mental Retardation Protein Expression in Neural Stem Cells Differentiated From Fragile X Syndrome Patient-Derived Induced Pluripotent Stem Cells.RNA-binding protein misregulation in microsatellite expansion disorders RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome

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Sequestration of DROSHA and DGCR8 by expanded CGG RNA repeats alters microRNA processing in fragile X-associated tremor/ataxia syndrome.

http://www.wikidata.org/entity/Q40221428

description

2013 nî lūn-bûn

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name

Sequestration of DROSHA and DG ...... ciated tremor/ataxia syndrome.

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type

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Sequestration of DROSHA and DG ...... ciated tremor/ataxia syndrome.

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Sequestration of DROSHA and DG ...... ciated tremor/ataxia syndrome.

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Sequestration of DROSHA and DG ...... ociated tremor/ataxia syndrome

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Adeline Page

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Chantal Sellier

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Fernande Freyermuth

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Flora Tassone

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Frank Ruffenach

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Matthew D Disney

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Paul J Hagerman

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Peter K Todd

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Ricardos Tabet

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Rob Willemsen

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P2860

MicroRNA-277 modulates the neurodegeneration caused by Fragile X premutation rCGG repeats

The nuclear RNase III Drosha initiates microRNA processing

The Microprocessor complex mediates the genesis of microRNAs

The Drosha-DGCR8 complex in primary microRNA processing

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

miRNAs are essential for survival and differentiation of newborn neurons but not for expansion of neural progenitors during early neurogenesis in the mouse embryonic neocortex

Intronic microRNA precursors that bypass Drosha processing

Cerebellar neurodegeneration in the absence of microRNAs

Structural insights into CUG repeats containing the 'stretched U-U wobble': implications for myotonic dystrophy

Crystal structures of CGG RNA repeats with implications for fragile X-associated tremor ataxia syndrome

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869-880

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10.1016/J.CELREP.2013.02.004

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3

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3

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Christelle Thibault

Nicolas Charlet

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235904997

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fragile X-associated tremor/ataxia syndrome

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3639429

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