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Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.

Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.

http://www.wikidata.org/entity/Q36295966

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MBNL and CELF proteins regulate alternative splicing of the skeletal muscle chloride channel CLCN1 Correction of ClC-1 splicing eliminates chloride channelopathy and myotonia in mouse models of myotonic dystrophy Progress toward therapy with antisense-mediated splicing modulation NMR spectroscopy and molecular dynamics simulation of r(CCGCUGCGG)₂ reveal a dynamic UU internal loop found in myotonic dystrophy type 1.Role of physiological ClC-1 Cl- ion channel regulation for the excitability and function of working skeletal muscle RNA and disease Mitochondria are linked to calcium stores in striated muscle by developmentally regulated tethering structures.Computational investigation of RNA CUG repeats responsible for myotonic dystrophy 1 Repression of nuclear CELF activity can rescue CELF-regulated alternative splicing defects in skeletal muscle models of myotonic dystrophy Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs DNA transfection of mammalian skeletal muscles using in vivo electroporation.Muscleblind1, but not Dmpk or Six5, contributes to a complex phenotype of muscular and motivational deficits in mouse models of myotonic dystrophy.Aberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy.Evoked myotonia can be "dialed-up" by increasing stimulus train length in myotonic dystrophy type 1.Chloride currents from the transverse tubular system in adult mammalian skeletal muscle fibers.Sarcolemmal-restricted localization of functional ClC-1 channels in mouse skeletal muscle Smaug/SAMD4A restores translational activity of CUGBP1 and suppresses CUG-induced myopathy.Triplet repeat RNA structure and its role as pathogenic agent and therapeutic target Muscleblind-Like 1 and Muscleblind-Like 3 Depletion Synergistically Enhances Myotonia by Altering Clc-1 RNA Translation.ClC1 chloride channel in myotonic dystrophy type 2 and ClC1 splicing in vitro.The muscle chloride channel ClC-1 is not directly regulated by intracellular ATP RNA interference targeting CUG repeats in a mouse model of myotonic dystrophy.Restricting calcium currents is required for correct fiber type specification in skeletal muscle.Transcriptional and post-transcriptional impact of toxic RNA in myotonic dystrophy.Myotonic dystrophy: therapeutic strategies for the future.Alternative splicing of RyR1 alters the efficacy of skeletal EC coupling.A Potent Inhibitor of Protein Sequestration by Expanded Triplet (CUG) Repeats that Shows Phenotypic Improvements in a Drosophila Model of Myotonic Dystrophy.Comparison of regulated passive membrane conductance in action potential-firing fast- and slow-twitch muscle Regulation of ClC-1 and KATP channels in action potential-firing fast-twitch muscle fibers Myotonic dystrophy mouse models: towards rational therapy development.Progress in therapeutic antisense applications for neuromuscular disorders.Most expression and splicing changes in myotonic dystrophy type 1 and type 2 skeletal muscle are shared with other muscular dystrophies.Epigenetic changes and non-coding expanded repeats.Targeting PKCθ in skeletal muscle and muscle diseases: good or bad?Developmental insights into the pathology of and therapeutic strategies for DM1: Back to the basics.Gene expression analysis in myotonic dystrophy: indications for a common molecular pathogenic pathway in DM1 and DM2.Muscle weakness in myotonic dystrophy associated with misregulated splicing and altered gating of Ca(V)1.1 calcium channel.Reversal of RNA dominance by displacement of protein sequestered on triplet repeat RNA.HTS-Compatible Patient-Derived Cell-Based Assay to Identify Small Molecule Modulators of Aberrant Splicing in Myotonic Dystrophy Type 1.Relaxing messages from the sarcolemma.

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Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.

http://www.wikidata.org/entity/Q36295966

description

2006 nî lūn-bûn

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2006年の論文

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2006年学术文章

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2006年学术文章

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2006年学术文章

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2006年学术文章

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2006年学术文章

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2006年學術文章

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2006年學術文章

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2006年學術文章

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name

Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.

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Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.

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type

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Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.

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Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.

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Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.

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Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy.

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The Journal of General Physiology

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Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy

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Ami Mankodi

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Charles A Thornton

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John D Lueck

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Robert T Dirksen

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P2860

Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches

Muscleblind proteins regulate alternative splicing

Molecular basis for decreased muscle chloride conductance in the myotonic goat

Myotonic dystrophy type 2 caused by a CCTG expansion in intron 1 of ZNF9

Membrane changes in cells from myotonia patients

Mutations in dominant human myotonia congenita drastically alter the voltage dependence of the CIC-1 chloride channel

Calcium currents in embryonic and neonatal mammalian skeletal muscle

Small-conductance calcium-activated potassium currents in mouse hyperexcitable denervated skeletal muscle

Chloride conductance in normal and myotonic muscle fibres and the action of monocarboxylic aromatic acids

Primary structure and functional expression of a developmentally regulated skeletal muscle chloride channel

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1

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129

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Maurice S. Swanson

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2006-12-11T00:00:00Z

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6640648

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