Sequestration of DROSHA and DGCR8 by expanded CGG RNA repeats alters microRNA processing in fragile X-associated tremor/ataxia syndrome. (Q40221428)

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25 February 2020

title

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

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fragile X-associated tremor/ataxia syndrome

25 February 2020

main subject

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5

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Hervé Moine

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Christelle Thibault

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Nicolas Charlet

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25 February 2020

author name string

Chantal Sellier

1

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25 February 2020

Fernande Freyermuth

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Ricardos Tabet

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Tuan Tran

4

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25 February 2020

Frank Ruffenach

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Violaine Alunni

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Adeline Page

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Flora Tassone

11

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25 February 2020

Rob Willemsen

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25 February 2020

Matthew D Disney

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Paul J Hagerman

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Peter K Todd

15

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25 February 2020

publication date

7 March 2013

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25 February 2020

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25 February 2020

volume

3

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25 February 2020

page(s)

869-880

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25 February 2020

issue

3

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A small molecule that targets r(CGG)(exp) and improves defects in fragile X-associated tremor ataxia syndrome

25 February 2020

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Sam68 sequestration and partial loss of function are associated with splicing alterations in FXTAS patients

22 September 2017

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Murine hippocampal neurons expressing Fmr1 gene premutations show early developmental deficits and late degeneration

22 September 2017

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

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Ectopic expression of CGG containing mRNA is neurotoxic in mammals

22 September 2017

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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

22 September 2017

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Altered brain microRNA biogenesis contributes to phenotypic deficits in a 22q11-deletion mouse model

22 September 2017

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Conditional loss of Dicer disrupts cellular and tissue morphogenesis in the cortex and hippocampus

22 September 2017

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Pur alpha binds to rCGG repeats and modulates repeat-mediated neurodegeneration in a Drosophila model of fragile X tremor/ataxia syndrome.

22 September 2017

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22 September 2017

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Regional FMRP deficits and large repeat expansions into the full mutation range in a new Fragile X premutation mouse model

22 September 2017

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DGCR8 is essential for microRNA biogenesis and silencing of embryonic stem cell self-renewal

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22 September 2017

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The structural basis of myotonic dystrophy from the crystal structure of CUG repeats

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Efficient processing of primary microRNA hairpins by Drosha requires flanking nonstructured RNA sequences

22 September 2017

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22 September 2017

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22 September 2017

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The fragile X syndrome repeats form RNA hairpins that do not activate the interferon-inducible protein kinase, PKR, but are cut by Dicer

22 September 2017

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The nuclear RNase III Drosha initiates microRNA processing

22 September 2017

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22 September 2017

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The FMR1 CGG repeat mouse displays ubiquitin-positive intranuclear neuronal inclusions; implications for the cerebellar tremor/ataxia syndrome

22 September 2017

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Fragile X premutation tremor/ataxia syndrome: molecular, clinical, and neuroimaging correlates

22 September 2017

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Accurate transcription initiation by RNA polymerase II in a soluble extract from isolated mammalian nuclei

22 September 2017

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Wnt1-cre-mediated conditional loss of Dicer results in malformation of the midbrain and cerebellum and failure of neural crest and dopaminergic differentiation in mice

22 September 2017

1 reference

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Genetic ablation of Dicer in adult forebrain neurons results in abnormal tau hyperphosphorylation and neurodegeneration.

17 June 2018

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Secondary structure and dynamics of the r(CGG) repeat in the mRNA of the fragile X mental retardation 1 (FMR1) gene

20 August 2018

1 reference

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RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS.

20 August 2018

1 reference

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CGG repeat length correlates with age of onset of motor signs of the fragile X-associated tremor/ataxia syndrome (FXTAS).

20 August 2018

1 reference

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FMR1 RNA within the intranuclear inclusions of fragile X-associated tremor/ataxia syndrome (FXTAS).

20 August 2018

1 reference

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Neuropathology of fragile X-associated tremor/ataxia syndrome (FXTAS).

20 August 2018

1 reference

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Protein composition of the intranuclear inclusions of FXTAS.

20 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3639429

Induction of inclusion formation and disruption of lamin A/C structure by premutation CGG-repeat RNA in human cultured neural cells

20 August 2018

1 reference

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RNA-mediated neurodegeneration caused by the fragile X premutation rCGG repeats in Drosophila

20 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3639429

Neuronal intranuclear inclusions in a new cerebellar tremor/ataxia syndrome among fragile X carriers

20 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3639429

Intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X.

20 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3639429

Fragile X males with unmethylated, full mutation trinucleotide repeat expansions have elevated levels of FMR1 messenger RNA.

20 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3639429

Ribonuclease dicer cleaves triplet repeat hairpins into shorter repeats that silence specific targets

20 August 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/23478018

12 December 2020

inferred from PubMed ID database lookup

Identifiers

DOI

10.1016/J.CELREP.2013.02.004

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/47ntsufzafc6lca2rgkqmeq6k4

30 July 2022

release_47ntsufzafc6lca2rgkqmeq6k4

Fatcat ID

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/47ntsufzafc6lca2rgkqmeq6k4

30 July 2022

https://api.fatcat.wiki/v0/release/47ntsufzafc6lca2rgkqmeq6k4

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30 July 2022

https://api.fatcat.wiki/v0/release/47ntsufzafc6lca2rgkqmeq6k4

1 reference

30 July 2022