Differential susceptibility of muscles to myotonia and force impairment in a mouse model of myotonic dystrophy. (Q48312174)

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title

Differential susceptibility of muscles to myotonia and force impairment in a mouse model of myotonic dystrophy (English)

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3

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author name string

Michelle Moyer

series ordinal

1

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Dara S Berger

series ordinal

2

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Erik Van Lunteren

series ordinal

4

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publication date

14 March 2011

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volume

43

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issue

6

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page(s)

818-827

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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.

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cites work

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An unstable triplet repeat in a gene related to myotonic muscular dystrophy

21 January 2018

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Myotonic dystrophy mutation: an unstable CTG repeat in the 3' untranslated region of the gene

21 January 2018

1 reference

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

21 January 2018

1 reference

Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy

21 January 2018

1 reference

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

21 January 2018

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Three proteins, MBNL, MBLL and MBXL, co-localize in vivo with nuclear foci of expanded-repeat transcripts in DM1 and DM2 cells.

21 January 2018

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MBNL1 is the primary determinant of focus formation and aberrant insulin receptor splicing in DM1

21 January 2018

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RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1

21 January 2018

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Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation

21 January 2018

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Cardiac tissue-specific repression of CELF activity disrupts alternative splicing and causes cardiomyopathy.

21 January 2018

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Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy

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A postnatal switch of CELF and MBNL proteins reprograms alternative splicing in the developing heart

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

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Coupled mRNA stabilization and translational silencing of cyclooxygenase-2 by a novel RNA binding protein, CUGBP2

21 January 2018

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RNA-dependent integrin alpha3 protein localization regulated by the Muscleblind-like protein MLP1

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Mice transgenic for the human myotonic dystrophy region with expanded CTG repeats display muscular and brain abnormalities

21 January 2018

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Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy

21 January 2018

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Expanded CTG repeats within the DMPK 3' UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophy

21 January 2018

1 reference

A muscleblind knockout model for myotonic dystrophy

21 January 2018

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

21 January 2018

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Overexpression of CUG triplet repeat-binding protein, CUGBP1, in mice inhibits myogenesis

21 January 2018

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Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy.

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Abnormal myotonic dystrophy protein kinase levels produce only mild myopathy in mice.

21 January 2018

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DM1 CTG expansions affect insulin receptor isoforms expression in various tissues of transgenic mice

21 January 2018

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

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Developmental control of the excitability of muscle: transplantation experiments on a myotonic mouse mutant

21 January 2018

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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

21 January 2018

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The myotonic mouse mutant ADR: physiological and histochemical properties of muscle.

21 January 2018

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Isotonic contractile impairment due to genetic CLC-1 chloride channel deficiency in myotonic mouse diaphragm muscle.

21 January 2018

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Motor-unit force potentiation in adult cats during a standard fatigue test

21 January 2018

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Reversal of RNA dominance by displacement of protein sequestered on triplet repeat RNA.

21 January 2018

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Central and peripheral respiratory electrophysiological studies in myotonic dystrophy

21 January 2018

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21 January 2018

Identifiers

DOI

10.1002/MUS.21988

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