(Q36895606)

16 August 2017

Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction (English)

1 reference

16 August 2017

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Christopher W Waters

1

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16 August 2017

Grigor Varuzhanyan

series ordinal

2

1 reference

16 August 2017

Robert J Talmadge

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3

1 reference

16 August 2017

Andrew A Voss

series ordinal

4

1 reference

16 August 2017

language of work or name

English

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publication date

13 May 2013

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Proceedings of the National Academy of Sciences of the United States of America

16 August 2017

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16 August 2017

110

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16 August 2017

22

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16 August 2017

9160-9165

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describes a project that uses

16 August 2017

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3670332/fullTextXML

inferred from PubMed Central ID database lookup

cites work

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1 reference

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CAG repeats mimic CUG repeats in the misregulation of alternative splicing

29 September 2017

1 reference

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Molecular characterization of skeletal muscle atrophy in the R6/2 mouse model of Huntington's disease

29 September 2017

1 reference

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Kir2.6 regulates the surface expression of Kir2.x inward rectifier potassium channels

29 September 2017

1 reference

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Chloride currents from the transverse tubular system in adult mammalian skeletal muscle fibers

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Sarcolemmal-restricted localization of functional ClC-1 channels in mouse skeletal muscle

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Huntington's disease: the current state of research with peripheral tissues.

29 September 2017

1 reference

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Muscleblind-like 2 (Mbnl2) -deficient mice as a model for myotonic dystrophy.

29 September 2017

1 reference

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Muscleblind-like 1 interacts with RNA hairpins in splicing target and pathogenic RNAs

29 September 2017

1 reference

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Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicing

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

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

29 September 2017

1 reference

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Ion permeation and selectivity in ClC-type chloride channels

29 September 2017

1 reference

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Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation.

29 September 2017

1 reference

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Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

29 September 2017

1 reference

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

29 September 2017

1 reference

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A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes.

29 September 2017

1 reference

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Molecular physiology of voltage-gated chloride channels

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Inactivation of muscle chloride channel by transposon insertion in myotonic mice

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

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

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Chloride conductance in mouse muscle is subject to post-transcriptional compensation of the functional Cl- channel 1 gene dosage.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Chloride currents across the membrane of mammalian skeletal muscle fibres.

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Use of hand-held dynamometry in the evaluation of lower limb muscle strength in people with Huntington's disease

27 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Clinical correlates of mitochondrial function in Huntington's disease muscle

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Early behavioral deficits in R6/2 mice suitable for use in preclinical drug testing

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Gene expression in Huntington's disease skeletal muscle: a potential biomarker

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Striatal potassium channel dysfunction in Huntington's disease transgenic mice

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

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

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington's disease mutation

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Myosin heavy chain isoform mRNA and protein levels after long-term paralysis

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Abnormal synaptic plasticity and impaired spatial cognition in mice transgenic for exon 1 of the human Huntington's disease mutation.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Formation of polyglutamine inclusions in non-CNS tissue

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

Complex I defect in muscle from patients with Huntington's disease.

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

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

4 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3670332

The influence of potassium and chloride ions on the membrane potential of single muscle fibres

4 September 2018

1 reference

12 December 2020

Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1

1 reference

12 December 2020

Rubidium block and rubidium permeability of the inward rectifier of frog skeletal muscle fibres

1 reference

12 December 2020

Inward rectification in the transverse tubular system of frog skeletal muscle studied with potentiometric dyes

1 reference

12 December 2020

Myotonic dystrophy in transgenic mice expressing an expanded CUG repeat

1 reference

12 December 2020

On the repetitive discharge in myotonic muscle fibres

1 reference

12 December 2020

Potassium depletion and sodium block of potassium currents under hyperpolarization in frog sartorius muscle

1 reference

12 December 2020

The chloride conductance of frog skeletal muscle

1 reference

12 December 2020

Identifiers

DOI

10.1073/PNAS.1220068110

1 reference

16 August 2017

0 references

1 reference

16 August 2017

1 reference