Reversal of RNA missplicing and myotonia after muscleblind overexpression in a mouse poly(CUG) model for myotonic dystrophy.
about
MBNL and CELF proteins regulate alternative splicing of the skeletal muscle chloride channel CLCN1RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense interventionMuscleblind-like 2-mediated alternative splicing in the developing brain and dysregulation in myotonic dystrophyRETRACTED: Alternative splicing produces high levels of noncoding isoforms of bHLH transcription factors during developmentCorrection of ClC-1 splicing eliminates chloride channelopathy and myotonia in mouse models of myotonic dystrophyMyotonic dystrophy: is a narrow focus obscuring the rest of the field?The unstable repeats--three evolving faces of neurological diseaseStructural insights into RNA recognition by the alternative-splicing regulator muscleblind-like MBNL1Modeling diseases of noncoding unstable repeat expansions using mutant pluripotent stem cells.RNA toxicity is a component of ataxin-3 degeneration in DrosophilaGenetic and chemical modifiers of a CUG toxicity model in DrosophilaThe expression analysis of Sfrs10 and Celf4 during mouse retinal developmentRNA and diseaseNKX2-5, a modifier of skeletal muscle pathology due to RNA toxicity.Evaluating the effects of CELF1 deficiency in a mouse model of RNA toxicityAge of onset of RNA toxicity influences phenotypic severity: evidence from an inducible mouse model of myotonic dystrophy (DM1).Therapeutics development in myotonic dystrophy type 1.Expanded CTG repeats within the DMPK 3' UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophyPKC inhibition ameliorates the cardiac phenotype in a mouse model of myotonic dystrophy type 1.Mechanistic determinants of MBNL activityCytoplasmic CUG RNA foci are insufficient to elicit key DM1 features.Repression of nuclear CELF activity can rescue CELF-regulated alternative splicing defects in skeletal muscle models of myotonic dystrophyDrosophila muscleblind is involved in troponin T alternative splicing and apoptosisRational and modular design of potent ligands targeting the RNA that causes myotonic dystrophy 2.Rational design of ligands targeting triplet repeating transcripts that cause RNA dominant disease: application to myotonic muscular dystrophy type 1 and spinocerebellar ataxia type 3.RNA gain-of-function in spinocerebellar ataxia type 8.Controlling the specificity of modularly assembled small molecules for RNA via ligand module spacing: targeting the RNAs that cause myotonic muscular dystrophyDevelopment of histone deacetylase inhibitors as therapeutics for neurological diseaseThe role of flexibility in the rational design of modularly assembled ligands targeting the RNAs that cause the myotonic dystrophiesAberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy.Myotonic dystrophies 1 and 2: complex diseases with complex mechanisms.The four Zn fingers of MBNL1 provide a flexible platform for recognition of its RNA binding elements.In vivo discovery of a peptide that prevents CUG-RNA hairpin formation and reverses RNA toxicity in myotonic dystrophy modelsMuscleblind-like 1 (Mbnl1) promotes insulin receptor exon 11 inclusion via binding to a downstream evolutionarily conserved intronic enhancer.Two high-throughput screening assays for aberrant RNA-protein interactions in myotonic dystrophy type 1.Design of a bioactive small molecule that targets the myotonic dystrophy type 1 RNA via an RNA motif-ligand database and chemical similarity searching.Rationally designed small molecules targeting the RNA that causes myotonic dystrophy type 1 are potently bioactive.RNA-binding protein Muscleblind-like 3 (MBNL3) disrupts myocyte enhancer factor 2 (Mef2) {beta}-exon splicing.RNA-protein interactions in unstable microsatellite diseases.MBNL proteins and their target RNAs, interaction and splicing regulation.
P2860
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P2860
Reversal of RNA missplicing and myotonia after muscleblind overexpression in a mouse poly(CUG) model for myotonic dystrophy.
description
2006 nî lūn-bûn
@nan
2006 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2006 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2006年の論文
@ja
2006年学术文章
@wuu
2006年学术文章
@zh-cn
2006年学术文章
@zh-hans
2006年学术文章
@zh-my
2006年学术文章
@zh-sg
2006年學術文章
@yue
name
Reversal of RNA missplicing and myotonia after muscleblind overexpression in a mouse poly
@nl
Reversal of RNA missplicing an ...... model for myotonic dystrophy.
@ast
Reversal of RNA missplicing an ...... model for myotonic dystrophy.
@en
type
label
Reversal of RNA missplicing and myotonia after muscleblind overexpression in a mouse poly
@nl
Reversal of RNA missplicing an ...... model for myotonic dystrophy.
@ast
Reversal of RNA missplicing an ...... model for myotonic dystrophy.
@en
prefLabel
Reversal of RNA missplicing and myotonia after muscleblind overexpression in a mouse poly
@nl
Reversal of RNA missplicing an ...... model for myotonic dystrophy.
@ast
Reversal of RNA missplicing an ...... model for myotonic dystrophy.
@en
P2093
P2860
P356
P1476
Reversal of RNA missplicing an ...... model for myotonic dystrophy.
@en
P2093
Charles A Thornton
Jihae Shin
Maurice S Swanson
Rahul N Kanadia
Stuart G Beattie
Thurman M Wheeler
P2860
P304
11748-11753
P356
10.1073/PNAS.0604970103
P407
P577
2006-07-24T00:00:00Z