Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy
about
The CELF family of RNA binding proteins is implicated in cell-specific and developmentally regulated alternative splicingMuscleblind proteins regulate alternative splicingInteraction of muscleblind, CUG-BP1 and hnRNP H proteins in DM1-associated aberrant IR splicingCalreticulin interacts with C/EBPalpha and C/EBPbeta mRNAs and represses translation of C/EBP proteinsMBNL and CELF proteins regulate alternative splicing of the skeletal muscle chloride channel CLCN1Control of a neuronal morphology program by an RNA-binding zinc finger protein, UnkemptFailure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophyMolecular basis for impaired muscle differentiation in myotonic dystrophyIn vivo co-localisation of MBNL protein with DMPK expanded-repeat transcriptsRNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense interventionAltered ribostasis: RNA-protein granules in degenerative disordersExpanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALSRNA-dependent integrin alpha3 protein localization regulated by the Muscleblind-like protein MLP1Comparative genomics and molecular dynamics of DNA repeats in eukaryotesElevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophyCorrection of ClC-1 splicing eliminates chloride channelopathy and myotonia in mouse models of myotonic dystrophyDefining early steps in mRNA transport: mutant mRNA in myotonic dystrophy type I is blocked at entry into SC-35 domains.HnRNP H inhibits nuclear export of mRNA containing expanded CUG repeats and a distal branch point sequence.Upregulated genes in sporadic, idiopathic pulmonary arterial hypertension.There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALSMuscle wasting in myotonic dystrophies: a model of premature agingRegulation of protein homeostasis in neurodegenerative diseases: the role of coding and non-coding genesRAN translation and frameshifting as translational challenges at simple repeats of human neurodegenerative disordersMyotonic dystrophy: is a narrow focus obscuring the rest of the field?Structural studies of CNG repeatsMechanisms of RNA-induced toxicity in CAG repeat disordersConverging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisEvolutionary conservation and expression of human RNA-binding proteins and their role in human genetic diseaseThe unstable repeats--three evolving faces of neurological diseaseA Caenorhabditis elegans locomotion phenotype caused by transgenic repeats of the hlh-17 promoter sequenceTriplet repeat-derived siRNAs enhance RNA-mediated toxicity in a Drosophila model for myotonic dystrophyStructural insights into RNA recognition by the alternative-splicing regulator muscleblind-like MBNL1Solution structure of the RNA binding domain in the human muscleblind-like protein 2Structure of dystrophia myotonica protein kinaseStructural insights into CUG repeats containing the 'stretched U-U wobble': implications for myotonic dystrophyAtomic resolution structure of CAG RNA repeats: structural insights and implications for the trinucleotide repeat expansion diseasesModeling diseases of noncoding unstable repeat expansions using mutant pluripotent stem cells.Drosophila as an In Vivo Model for Human Neurodegenerative DiseaseIdentification of variants in MBNL1 in patients with a myotonic dystrophy-like phenotypeRNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1
P2860
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P2860
Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy
description
2000 nî lūn-bûn
@nan
2000 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի սեպտեմբերին հրատարակված գիտական հոդված
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2000年の論文
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2000年学术文章
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2000年学术文章
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2000年学术文章
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2000年学术文章
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2000年学术文章
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2000年學術文章
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name
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@ast
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@en
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@en-gb
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@nl
type
label
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@ast
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@en
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@en-gb
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@nl
prefLabel
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@ast
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@en
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@en-gb
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@nl
P2093
P2860
P356
P1433
P1476
Recruitment of human musclebli ...... ciated with myotonic dystrophy
@en
P2093
C A Thornton
C R Urbinati
J W Miller
M G Stenberg
M S Swanson
P Teng-Umnuay
P2860
P304
P356
10.1093/EMBOJ/19.17.4439
P407
P577
2000-09-01T00:00:00Z