The RNA-binding protein Staufen1 is increased in DM1 skeletal muscle and promotes alternative pre-mRNA splicing
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Myotonic dystrophy: is a narrow focus obscuring the rest of the field?Staufen1 dimerizes through a conserved motif and a degenerate dsRNA-binding domain to promote mRNA decayReport on the 3rd Ottawa International Conference on Neuromuscular Biology, Disease and Therapy - September 24-26, 2015, Ottawa, Canada.Age of onset of RNA toxicity influences phenotypic severity: evidence from an inducible mouse model of myotonic dystrophy (DM1).RNA-protein interactions in unstable microsatellite diseases.RNA-binding protein misregulation in microsatellite expansion disordersIncreased autophagy and apoptosis contribute to muscle atrophy in a myotonic dystrophy type 1 Drosophila model.Reduction of toxic RNAs in myotonic dystrophies type 1 and type 2 by the RNA helicase p68/DDX5Nuclear retention of full-length HTT RNA is mediated by splicing factors MBNL1 and U2AF65Staufen1 Regulates Multiple Alternative Splicing Events either Positively or Negatively in DM1 Indicating Its Role as a Disease ModifierMBNL Sequestration by Toxic RNAs and RNA Misprocessing in the Myotonic Dystrophy BrainStaufen1s role as a splicing factor and a disease modifier in Myotonic Dystrophy Type I.Antisense oligonucleotides: rising stars in eliminating RNA toxicity in myotonic dystrophy.Staufen1 impairs stress granule formation in skeletal muscle cells from myotonic dystrophy type 1 patients.STAU1 binding 3' UTR IRAlus complements nuclear retention to protect cells from PKR-mediated translational shutdownProgressive impairment of muscle regeneration in muscleblind-like 3 isoform knockout mice.Posttranscriptional regulation of insulin family ligands and receptorsGenome-wide analysis of Staufen-associated mRNAs identifies secondary structures that confer target specificityIdentifying proteins that bind to specific RNAs - focus on simple repeat expansion diseases.Compound loss of muscleblind-like function in myotonic dystrophy.Treatment of type 1 myotonic dystrophy by engineering site-specific RNA endonucleases that target (CUG)(n) repeats.Novel Roles for Staufen1 in Embryonal and Alveolar Rhabdomyosarcoma via c-myc-dependent and -independent events.Developmental insights into the pathology of and therapeutic strategies for DM1: Back to the basics.Molecular mechanisms in DM1 - a focus on foci.Muscle-specific expression of the RNA-binding protein Staufen1 induces progressive skeletal muscle atrophy via regulation of phosphatase tensin homolog.Cell cycle-dependent regulation of the RNA-binding protein Staufen1.Can Human Pluripotent Stem Cell-Derived Cardiomyocytes Advance Understanding of Muscular Dystrophies?DDX6 regulates sequestered nuclear CUG-expanded DMPK-mRNA in dystrophia myotonica type 1.Staufen1 senses overall transcript secondary structure to regulate translationChronic 5-Aminoimidazole-4-Carboxamide-1-β-d-Ribofuranoside Treatment Induces Phenotypic Changes in Skeletal Muscle, but Does Not Improve Disease Outcomes in the R6/2 Mouse Model of Huntington's Disease.The RNA-binding protein Staufen1 impairs myogenic differentiation via a c-myc-dependent mechanism.Correction of GSK3β at young age prevents muscle pathology in mice with myotonic dystrophy type 1.CLIPing Staufen to secondary RNA structures: size and location matter!Misregulation of calcium-handling proteins promotes hyperactivation of calcineurin-NFAT signaling in skeletal muscle of DM1 mice.Cytoplasmic functions of long noncoding RNAs.Diversification of the muscle proteome through alternative splicing.Abnormalities in Skeletal Muscle Myogenesis, Growth, and Regeneration in Myotonic Dystrophy.Cells of Matter-In Vitro Models for Myotonic Dystrophy.The crystal structure of Staufen1 in complex with a physiological RNA sheds light on substrate selectivity
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P2860
The RNA-binding protein Staufen1 is increased in DM1 skeletal muscle and promotes alternative pre-mRNA splicing
description
2012 nî lūn-bûn
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2012 թուականի Մարտին հրատարակուած գիտական յօդուած
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2012 թվականի մարտին հրատարակված գիտական հոդված
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2012年の論文
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2012年論文
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2012年論文
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2012年論文
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2012年論文
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2012年論文
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2012年论文
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name
The RNA-binding protein Staufe ...... alternative pre-mRNA splicing
@ast
The RNA-binding protein Staufe ...... alternative pre-mRNA splicing
@en
type
label
The RNA-binding protein Staufe ...... alternative pre-mRNA splicing
@ast
The RNA-binding protein Staufe ...... alternative pre-mRNA splicing
@en
prefLabel
The RNA-binding protein Staufe ...... alternative pre-mRNA splicing
@ast
The RNA-binding protein Staufe ...... alternative pre-mRNA splicing
@en
P2093
P2860
P356
P1476
The RNA-binding protein Staufe ...... alternative pre-mRNA splicing
@en
P2093
Aymeric Ravel-Chapuis
Bernard J Jasmin
Guy Bélanger
Jocelyn Côté
Luc DesGroseillers
Mani S Mahadevan
Ramesh S Yadava
P2860
P304
P356
10.1083/JCB.201108113
P407
P577
2012-03-01T00:00:00Z