Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy. (Q36295966)

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22 December 2019

title

Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy (English)

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22 December 2019

3

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22 December 2019

author name string

John D Lueck

1

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22 December 2019

Ami Mankodi

2

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22 December 2019

Charles A Thornton

4

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22 December 2019

Robert T Dirksen

5

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22 December 2019

publication date

11 December 2006

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The Journal of General Physiology

22 December 2019

published in

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22 December 2019

volume

129

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22 December 2019

issue

1

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22 December 2019

page(s)

79-94

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Reversal of RNA missplicing and myotonia after muscleblind overexpression in a mouse poly(CUG) model for myotonic dystrophy

22 December 2019

cites work

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Regulated sarcolemmal localization of the muscle-specific ClC-1 chloride channel.

30 September 2017

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Muscleblind proteins regulate alternative splicing

30 September 2017

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The role of the carboxyl terminus in ClC chloride channel function

30 September 2017

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A muscleblind knockout model for myotonic dystrophy

30 September 2017

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Expression and distribution of a small-conductance calcium-activated potassium channel (SK3) protein in skeletal muscles from myotonic muscular dystrophy patients and congenital myotonic mice

30 September 2017

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The myotonia congenita mutation A331T confers a novel hyperpolarization-activated gate to the muscle chloride channel ClC-1.

30 September 2017

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Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy

30 September 2017

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Myotonia caused by mutations in the muscle chloride channel gene CLCN1.

30 September 2017

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

30 September 2017

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Muscleblind localizes to nuclear foci of aberrant RNA in myotonic dystrophy types 1 and 2.

30 September 2017

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

30 September 2017

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Skeletal muscle sodium channel gating in mice deficient in myotonic dystrophy protein kinase

30 September 2017

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Patch-clamp recording of charge movement, Ca2+ current, and Ca2+ transients in adult skeletal muscle fibers

30 September 2017

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Regulation of the human skeletal muscle chloride channel hClC-1 by protein kinase C.

30 September 2017

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Permeation and block of the skeletal muscle chloride channel, ClC-1, by foreign anions

30 September 2017

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Expansion of a CUG trinucleotide repeat in the 3' untranslated region of myotonic dystrophy protein kinase transcripts results in nuclear retention of transcripts

30 September 2017

1 reference

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Expression of chloride channel 1 mRNA in cultured myogenic cells: a marker of myotube maturation

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2151606

Molecular basis for decreased muscle chloride conductance in the myotonic goat

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2151606

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

30 September 2017

1 reference

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Low single channel conductance of the major skeletal muscle chloride channel, ClC-1.

30 September 2017

1 reference

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Foci of trinucleotide repeat transcripts in nuclei of myotonic dystrophy cells and tissues

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2151606

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

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2151606

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

30 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2151606

Transient weakness and altered membrane characteristic in recessive generalized myotonia (Becker)

30 September 2017

1 reference

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Membrane changes in cells from myotonia patients

30 September 2017

1 reference

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Calcium currents in embryonic and neonatal mammalian skeletal muscle

30 September 2017

1 reference

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30 September 2017

Muscle chloride channels

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2151606

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

30 September 2017

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Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3' end of a transcript encoding a protein kinase family member.

30 September 2017

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Enhanced dihydropyridine receptor channel activity in the presence of ryanodine receptor.

30 September 2017

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Chloride currents across the membrane of mammalian skeletal muscle fibres

29 June 2018

1 reference

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Truncated ClC-1 mRNA in myotonic dystrophy exerts a dominant-negative effect on the Cl current

29 June 2018

1 reference

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Role of phosphorylation and physiological state in the regulation of the muscular chloride channel ClC-1: a voltage-clamp study on isolated M. interosseus fibers.

22 September 2018

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Development of electrical myotonia in the ADR mouse: role of chloride conductance in myotubes and neonatal animals.

22 September 2018

1 reference

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Role of innervation, excitability, and myogenic factors in the expression of the muscular chloride channel ClC-1. A study on normal and myotonic muscle

22 September 2018

1 reference

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The variance of sodium current fluctuations at the node of Ranvier

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2151606

Expression of apamin receptor in muscles of patients with myotonic muscular dystrophy

22 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2151606

Myotonic dystrophy in transgenic mice expressing an expanded CUG repeat

22 September 2018

1 reference

12 December 2020

Decreased levels of myotonic dystrophy protein kinase (DMPK) and delayed differentiation in human myotonic dystrophy myoblasts

1 reference

12 December 2020

The pathophysiology of myotonia produced by aromatic carboxylic acids

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12 December 2020

Ionic currents in slow twitch skeletal muscle in the rat

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12 December 2020

Membrane ionic conductances in normal and denervated skeletal muscle of the rat during development

1 reference

12 December 2020

Nonstationary noise analysis and application to patch clamp recordings

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12 December 2020

Possible role of apamin-sensitive K+ channels in myotonic dystrophy

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12 December 2020

Effect of transverse-tubular chloride conductance on excitability in skinned skeletal muscle fibres of rat and toad

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12 December 2020

Identifiers

DOI

10.1085/JGP.200609635

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22 December 2019

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22 December 2019

PubMed publication ID

17158949

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